00
Correct
00
Incorrect
00 : 00 : 00
Session Time
00 : 00
Average Question Time ( Secs)
  • Question 1 - Which one of the following statements about blood clotting is false? ...

    Correct

    • Which one of the following statements about blood clotting is false?

      Your Answer: Administration of aprotinin during liver transplantation surgery prolongs survival

      Explanation:

      Aprotinin, which decreases bleeding by inhibiting fibrinolysis, was taken off the market in 2007 due to its link to higher mortality rates. Vitamin K-dependent protein C may actually increase the risk of thrombosis in the initial stages of warfarin treatment.

      The Coagulation Cascade: Two Pathways to Fibrin Formation

      The coagulation cascade is a complex process that leads to the formation of a blood clot. There are two pathways that can lead to fibrin formation: the intrinsic pathway and the extrinsic pathway. The intrinsic pathway involves components that are already present in the blood and has a minor role in clotting. It is initiated by subendothelial damage, such as collagen, which leads to the formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and Factor 12. This complex activates Factor 11, which in turn activates Factor 9. Factor 9, along with its co-factor Factor 8a, forms the tenase complex, which activates Factor 10.

      The extrinsic pathway, on the other hand, requires tissue factor released by damaged tissue. This pathway is initiated by tissue damage, which leads to the binding of Factor 7 to tissue factor. This complex activates Factor 9, which works with Factor 8 to activate Factor 10. Both pathways converge at the common pathway, where activated Factor 10 causes the conversion of prothrombin to thrombin. Thrombin hydrolyses fibrinogen peptide bonds to form fibrin and also activates factor 8 to form links between fibrin molecules.

      Finally, fibrinolysis occurs, which is the process of clot resorption. Plasminogen is converted to plasmin to facilitate this process. It is important to note that certain factors are involved in both pathways, such as Factor 10, and that some factors are vitamin K dependent, such as Factors 2, 7, 9, and 10. The intrinsic pathway can be assessed by measuring the activated partial thromboplastin time (APTT), while the extrinsic pathway can be assessed by measuring the prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology And Oncology
      7.5
      Seconds
  • Question 2 - A 49-year-old female presents to her family physician with complaints of post-coital pain....

    Incorrect

    • A 49-year-old female presents to her family physician with complaints of post-coital pain. She initially attributed it to her age, but lately, she has been experiencing a constant dull pain in her pelvis. Additionally, she reports having a foul-smelling discharge from her vagina. Her medical and surgical history is unremarkable, but she mentions having multiple sexual partners during her teenage years and twenties. She has been smoking ten cigarettes a day for the past decade and does not consume alcohol. During the examination, the doctor discovers an irregular mass on her cervix. What is the primary mechanism behind the most significant risk factor for this patient's condition?

      Your Answer: Having multiple sexual partners increase the risk of getting HIV which then expresses viral proteins leading to cervical dysplasia and carcinoma

      Correct Answer: Human papillomavirus 16 and 18 produces oncoproteins which causes inhibition of the tumor suppressor genes causing cervical carcinoma

      Explanation:

      The patient is displaying typical signs and symptoms of cervical carcinoma, with a constant dull pelvic pain indicating possible invasion of pelvic structures and nerves. The strongest risk factor for this patient is having had multiple sexual partners at a young age, which increases the likelihood of being infected with the human papillomavirus.

      1: Multiple sexual partners are the strongest risk factor for cervical carcinoma due to the increased chance of contracting the human papillomavirus, specifically the 16 and 18 viral strains that inhibit the tumor suppressor genes p53 and RB, triggering carcinogenesis.
      2: While cigarette smoking can have an oncogenic effect, it is not the primary risk factor in this case.
      3: HIV is a risk factor for cervical carcinoma, but it is less common than the human papillomavirus.
      4: The human papillomavirus is the primary risk factor, but it does not activate oncogenes. Instead, it inhibits tumor suppressor genes.
      5: Age alone is not a risk factor for cervical carcinoma. However, an older person who has been exposed to the human papillomavirus may have a higher risk due to the longer exposure time for the virus to induce carcinogenesis via the inhibition of tumor suppressor genes.

      HPV Infection and Cervical Cancer

      Human papillomavirus (HPV) infection is the primary risk factor for cervical cancer, with subtypes 16, 18, and 33 being the most carcinogenic. Other common subtypes, such as 6 and 11, are associated with genital warts but are not carcinogenic. When endocervical cells become infected with HPV, they may undergo changes that lead to the development of koilocytes. These cells have distinct characteristics, including an enlarged nucleus, irregular nuclear membrane contour, hyperchromasia (darker staining of the nucleus), and a perinuclear halo. These changes are important diagnostic markers for cervical cancer and can be detected through Pap smears or other screening methods. Early detection and treatment of HPV infection and cervical cancer can greatly improve outcomes and reduce the risk of complications.

    • This question is part of the following fields:

      • Haematology And Oncology
      9.5
      Seconds
  • Question 3 - A 43-year-old woman comes to your clinic complaining of unexplained weight gain, cold...

    Correct

    • A 43-year-old woman comes to your clinic complaining of unexplained weight gain, cold intolerance, and fatigue. You suspect hypothyroidism and decide to conduct a test on her serum levels of thyroid stimulating hormone (TSH) and free thyroxine (T4). The release of thyroid hormone is regulated through a negative feedback mechanism. Which of the following is not regulated through a negative feedback mechanism?

      Your Answer: Clotting cascade

      Explanation:

      The clotting cascade is an example of a positive feedback mechanism, where the presence of clotting factors attracts further clotting factors until a functioning clot is formed. On the other hand, blood sugar, blood pressure, and cortisol are controlled via negative feedback mechanisms. When blood sugar rises, insulin is released to transport glucose into cells, lowering blood sugar. When BP is low, the RAAS is activated to increase BP through vasoconstriction and retention of salt and water. Cortisol is released in response to ACTH, which is inhibited by high levels of cortisol through negative feedback on the hypothalamus and anterior pituitary.

      The Coagulation Cascade: Two Pathways to Fibrin Formation

      The coagulation cascade is a complex process that leads to the formation of a blood clot. There are two pathways that can lead to fibrin formation: the intrinsic pathway and the extrinsic pathway. The intrinsic pathway involves components that are already present in the blood and has a minor role in clotting. It is initiated by subendothelial damage, such as collagen, which leads to the formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and Factor 12. This complex activates Factor 11, which in turn activates Factor 9. Factor 9, along with its co-factor Factor 8a, forms the tenase complex, which activates Factor 10.

      The extrinsic pathway, on the other hand, requires tissue factor released by damaged tissue. This pathway is initiated by tissue damage, which leads to the binding of Factor 7 to tissue factor. This complex activates Factor 9, which works with Factor 8 to activate Factor 10. Both pathways converge at the common pathway, where activated Factor 10 causes the conversion of prothrombin to thrombin. Thrombin hydrolyses fibrinogen peptide bonds to form fibrin and also activates factor 8 to form links between fibrin molecules.

      Finally, fibrinolysis occurs, which is the process of clot resorption. Plasminogen is converted to plasmin to facilitate this process. It is important to note that certain factors are involved in both pathways, such as Factor 10, and that some factors are vitamin K dependent, such as Factors 2, 7, 9, and 10. The intrinsic pathway can be assessed by measuring the activated partial thromboplastin time (APTT), while the extrinsic pathway can be assessed by measuring the prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology And Oncology
      6.9
      Seconds
  • Question 4 - During a placement in general practice, a 56-year-old woman comes in with new...

    Incorrect

    • During a placement in general practice, a 56-year-old woman comes in with new nipple discharge and skin dimpling over her breast. The GP conducts a breast examination, including the lymph nodes surrounding the area. Which lymph nodes receive the most breast lymph?

      Your Answer: Parasternal lymph nodes

      Correct Answer: Axilliary lymph nodes

      Explanation:

      The lymphatic system of the breast is responsible for draining excess fluid and waste products. Lymph from the upper outer quadrant of the breast drains to the axillary lymph nodes, while lymph from the inner quadrants drains to the parasternal lymph nodes. Additionally, some lymph from the lower quadrants drains to the inferior phrenic lymph nodes.

      Lymphatic drainage is the process by which lymphatic vessels carry lymph, a clear fluid containing white blood cells, away from tissues and organs and towards lymph nodes. The lymphatic vessels that drain the skin and follow venous drainage are called superficial lymphatic vessels, while those that drain internal organs and structures follow the arteries and are called deep lymphatic vessels. These vessels eventually lead to lymph nodes, which filter and remove harmful substances from the lymph before it is returned to the bloodstream.

      The lymphatic system is divided into two main ducts: the right lymphatic duct and the thoracic duct. The right lymphatic duct drains the right side of the head and right arm, while the thoracic duct drains everything else. Both ducts eventually drain into the venous system.

      Different areas of the body have specific primary lymph node drainage sites. For example, the superficial inguinal lymph nodes drain the anal canal below the pectinate line, perineum, skin of the thigh, penis, scrotum, and vagina. The deep inguinal lymph nodes drain the glans penis, while the para-aortic lymph nodes drain the testes, ovaries, kidney, and adrenal gland. The axillary lymph nodes drain the lateral breast and upper limb, while the internal iliac lymph nodes drain the anal canal above the pectinate line, lower part of the rectum, and pelvic structures including the cervix and inferior part of the uterus. The superior mesenteric lymph nodes drain the duodenum and jejunum, while the inferior mesenteric lymph nodes drain the descending colon, sigmoid colon, and upper part of the rectum. Finally, the coeliac lymph nodes drain the stomach.

    • This question is part of the following fields:

      • Haematology And Oncology
      5.9
      Seconds
  • Question 5 - A 52-year-old male comes to see you with complaints of altered sensation in...

    Correct

    • A 52-year-old male comes to see you with complaints of altered sensation in his arms. He reports that he first noticed changes four months ago when he could no longer feel the sleeves of his shirts on his arms. This has now progressed to numbness and tingling in his forearms, which started one month ago. He has no medical history and is not taking any medications. You inquire about his diet as you suspect a possible vitamin deficiency.

      What vitamin deficiency is the most likely cause of his symptoms?

      Your Answer: Vitamin B12

      Explanation:

      The initial impact of vitamin B12 deficiency is typically on the dorsal column, causing impairment in joint position and vibration perception before the onset of distal paraesthesia.

      Vitamin B12 is essential for the development of red blood cells and the maintenance of the nervous system. It is absorbed through the binding of intrinsic factor, which is secreted by parietal cells in the stomach, and actively absorbed in the terminal ileum. A deficiency in vitamin B12 can be caused by pernicious anaemia, post gastrectomy, a vegan or poor diet, disorders or surgery of the terminal ileum, Crohn’s disease, or metformin use.

      Symptoms of vitamin B12 deficiency include macrocytic anaemia, a sore tongue and mouth, neurological symptoms, and neuropsychiatric symptoms such as mood disturbances. The dorsal column is usually affected first, leading to joint position and vibration issues before distal paraesthesia.

      Management of vitamin B12 deficiency involves administering 1 mg of IM hydroxocobalamin three times a week for two weeks, followed by once every three months if there is no neurological involvement. If a patient is also deficient in folic acid, it is important to treat the B12 deficiency first to avoid subacute combined degeneration of the cord.

    • This question is part of the following fields:

      • Haematology And Oncology
      5.9
      Seconds
  • Question 6 - A 45-year-old man with a history of chronic alcoholism presents to his GP...

    Incorrect

    • A 45-year-old man with a history of chronic alcoholism presents to his GP with complaints of fatigue and breathlessness upon exertion. During examination, no splenomegaly was observed. A peripheral smear revealed microcytic red blood cells with basophilic stippling. A bone marrow biopsy showed an increased uptake of Prussian blue. The patient's iron levels and transferrin saturation were high, while both mean corpuscular volume (MCV) and mean corpuscular hemoglobin were low. Laboratory results showed a hemoglobin level of 95 g/L (normal range for males: 135-180 g/L), platelets of 200 * 109/L (normal range: 150-400 * 109/L), and WBC of 7.0 * 109/L (normal range: 4.0-11.0 * 109/L). The patient's ferritin level was 300 ng/mL (normal range: 20-230 ng/mL), and his vitamin B12 level was 400 ng/L (normal range: 200-900 ng/L). What is the most likely disease that the patient is suffering from?

      Your Answer: Spherocytosis

      Correct Answer: Sideroblastic anaemia

      Explanation:

      The correct diagnosis for the patient is sideroblastic anaemia, which is characterized by hypochromic microcytic anaemia, high levels of ferritin iron and transferrin saturation, and basophilic stippling of red blood cells. This condition is caused by vitamin B6 deficiency due to frequent alcohol consumption, leading to abnormal heme production. The peripheral smear shows basophilic stippling of red blood cells, and there is iron overload causing iron deposition in the bone marrow, observed as increased staining with Prussian blue.

      Anaemia of chronic disease, iron deficiency anaemia, and aplastic anaemia are incorrect diagnoses. Anaemia of chronic disease is usually normocytic normochromic and has significantly low levels of folate, B12, and iron while ferritin is high. Iron deficiency anaemia may be microcytic hypochromic, but serum iron, ferritin, and transferrin levels would be reduced. Aplastic anaemia presents with pancytopenia and is rarely found in the given age group.

      Understanding Sideroblastic Anaemia

      Sideroblastic anaemia is a medical condition that occurs when red blood cells fail to produce enough haem, which is partly synthesized in the mitochondria. This results in the accumulation of iron in the mitochondria, forming a ring around the nucleus known as a ring sideroblast. The condition can be either congenital or acquired.

      The congenital cause of sideroblastic anaemia is delta-aminolevulinate synthase-2 deficiency. On the other hand, acquired causes include myelodysplasia, alcohol, lead, and anti-TB medications.

      To diagnose sideroblastic anaemia, doctors may conduct a full blood count, iron studies, and a blood film. The results may show hypochromic microcytic anaemia, high ferritin, high iron, high transferrin saturation, and basophilic stippling of red blood cells. A bone marrow test may also be done, and Prussian blue staining can reveal ringed sideroblasts.

      Management of sideroblastic anaemia is mainly supportive, and treatment focuses on addressing any underlying cause. Pyridoxine may also be prescribed to help manage the condition.

      In summary, sideroblastic anaemia is a condition that affects the production of haem in red blood cells, leading to the accumulation of iron in the mitochondria. It can be congenital or acquired, and diagnosis involves various tests. Treatment is mainly supportive, and addressing any underlying cause is crucial.

    • This question is part of the following fields:

      • Haematology And Oncology
      8.3
      Seconds
  • Question 7 - Samantha is a 42-year-old woman who has presented with new-onset urinary retention over...

    Incorrect

    • Samantha is a 42-year-old woman who has presented with new-onset urinary retention over the past 8 hours. She was previously diagnosed with non-Hodgkin's lymphoma and is currently undergoing chemotherapy treatment for this. She notes that prior to this, her urine had a reddish-tinge.

      A 3-way catheter was inserted and blood-stained urine with clots was seen within the catheter bag. Urinalysis showed significant blood but no leukocytes or nitrites were seen. A cystoscopy performed did not show any masses and biopsies taken did not show any malignancy. It was felt that this was a likely side effect of one of these chemotherapy agents.

      What is the underlying mechanism of action of the culprit chemotherapy agent?

      Your Answer: Inhibits microtubule formation

      Correct Answer: Promotes cross-linking of DNA

      Explanation:

      The chemotherapy regime R-CHOP, which is likely being used to manage the patient’s non-Hodgkin’s lymphoma, includes cyclophosphamide, a drug that functions as an alkylating agent and promotes cross-linking of DNA. This can lead to haemorrhagic cystitis, which is likely the cause of the patient’s haematuria. Other drugs in the regime have different mechanisms of action, such as inhibition of microtubule formation with vincristine, inhibition of topoisomerase II and DNA/RNA synthesis with doxorubicin, and monoclonal antibody targeting of CD20 with rituximab. Pyrimidine analogues like 5-fluorouracil, which block thymidylate synthase and induce cell cycle arrest and apoptosis, are not commonly used in the management of non-Hodgkin’s lymphoma.

      Cytotoxic agents are drugs that are used to kill cancer cells. There are several types of cytotoxic agents, each with their own mechanism of action and potential adverse effects. Alkylating agents, such as cyclophosphamide, work by causing cross-linking in DNA. However, they can also cause haemorrhagic cystitis, myelosuppression, and transitional cell carcinoma. Cytotoxic antibiotics, like bleomycin and anthracyclines, degrade preformed DNA and stabilize DNA-topoisomerase II complex, respectively. However, they can also cause lung fibrosis and cardiomyopathy. Antimetabolites, such as methotrexate and fluorouracil, inhibit dihydrofolate reductase and thymidylate synthesis, respectively. However, they can also cause myelosuppression, mucositis, and liver or lung fibrosis. Drugs that act on microtubules, like vincristine and docetaxel, inhibit the formation of microtubules and prevent microtubule depolymerisation & disassembly, respectively. However, they can also cause peripheral neuropathy, myelosuppression, and paralytic ileus. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I, which prevents relaxation of supercoiled DNA. However, they can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin and hydroxyurea, cause cross-linking in DNA and inhibit ribonucleotide reductase, respectively. However, they can also cause ototoxicity, peripheral neuropathy, hypomagnesaemia, and myelosuppression.

    • This question is part of the following fields:

      • Haematology And Oncology
      6.9
      Seconds
  • Question 8 - What type of cell is found in higher quantities in the blood sample...

    Incorrect

    • What type of cell is found in higher quantities in the blood sample of an individual who has a viral infection?

      Your Answer: Neutrophils

      Correct Answer: Lymphocytes

      Explanation:

      Blood Cell Types and Their Presence in Various Disorders

      Lymphocytes are a type of blood cell that can be found in higher numbers during viral infections. Eosinophils, on the other hand, are present in response to allergies, drug reactions, or infections caused by flatworms and strongyloides. Monocytes are another type of blood cell that can be found in disorders such as EBV infection, CMML, and other atypical infections. Neutrophils are present in bacterial infections or in disorders such as CML or AML where their more immature blastoid form is seen. Lastly, platelets can be increased in infections, iron deficiency, or myeloproliferative disorders.

      In summary, different types of blood cells can indicate various disorders or infections. By analyzing the presence of these cells in the blood, doctors can better diagnose and treat patients. It is important to note that the presence of these cells alone is not enough to make a diagnosis, and further testing may be necessary.

    • This question is part of the following fields:

      • Haematology And Oncology
      6.1
      Seconds
  • Question 9 - A 58-year-old woman presents to her GP with a 4-week history of abdominal...

    Incorrect

    • A 58-year-old woman presents to her GP with a 4-week history of abdominal pain, fatigue and bruising. After various investigations, she is diagnosed with chronic myeloid leukaemia. What is the probable pathophysiology responsible for her abdominal pain?

      Your Answer: Duodenal ulcer

      Correct Answer: Splenomegaly

      Explanation:

      Massive splenomegaly can be a symptom of chronic myeloid leukaemia (CML), which is the known diagnosis of this woman. Left-sided swelling, increased tendency to bruise or bleed, and abdominal pain may also be present. However, a duodenal ulcer is more likely to cause indigestion and is not commonly associated with CML. While hepatomegaly may occur in CML, it is less common and less marked than splenomegaly. Large bowel obstruction is not typically associated with CML, but may be a presenting symptom of undiagnosed colorectal cancer. Although splenic rupture can cause abdominal pain, it is more likely to lead to an acute presentation due to complications of acute intra-abdominal bleeding.

      Understanding Chronic Myeloid Leukaemia and its Management

      Chronic myeloid leukaemia (CML) is a type of cancer that affects the blood and bone marrow. It is characterized by the presence of the Philadelphia chromosome in more than 95% of patients. This chromosome is formed due to a translocation between chromosomes 9 and 22, resulting in the fusion of the ABL proto-oncogene and the BCR gene. The resulting BCR-ABL gene produces a fusion protein that has excessive tyrosine kinase activity.

      CML typically affects individuals between the ages of 60-70 years and presents with symptoms such as anaemia, weight loss, sweating, and splenomegaly. The condition is also associated with an increase in granulocytes at different stages of maturation and thrombocytosis. In some cases, CML may undergo blast transformation, leading to acute myeloid leukaemia (AML) or acute lymphoblastic leukaemia (ALL).

      The management of CML involves various treatment options, including imatinib, which is considered the first-line treatment. Imatinib is an inhibitor of the tyrosine kinase associated with the BCR-ABL defect and has a very high response rate in chronic phase CML. Other treatment options include hydroxyurea, interferon-alpha, and allogeneic bone marrow transplant. With proper management, individuals with CML can lead a normal life.

    • This question is part of the following fields:

      • Haematology And Oncology
      6.2
      Seconds
  • Question 10 - A 25-year-old male patient arrives with blunt abdominal trauma and a suspected splenic...

    Incorrect

    • A 25-year-old male patient arrives with blunt abdominal trauma and a suspected splenic bleed. The medical team initiates an infusion of tranexamic acid. What is the mechanism of action of tranexamic acid?

      Your Answer:

      Correct Answer: Inhibition of plasmin

      Explanation:

      The prevention of fibrin degradation is achieved by the inhibition of plasmin through the use of tranexamic acid.

      Understanding Tranexamic Acid

      Tranexamic acid is a synthetic derivative of lysine that acts as an antifibrinolytic. Its primary function is to bind to lysine receptor sites on plasminogen or plasmin, preventing plasmin from degrading fibrin. This medication is commonly prescribed to treat menorrhagia.

      In addition to its use in treating menorrhagia, tranexamic acid has been investigated for its role in trauma. The CRASH 2 trial found that administering tranexamic acid within the first 3 hours of bleeding trauma can be beneficial. In cases of major haemorrhage, tranexamic acid is given as an IV bolus followed by an infusion.

      Ongoing research is also exploring the potential of tranexamic acid in treating traumatic brain injury. Overall, tranexamic acid is a medication with important applications in managing bleeding disorders and trauma.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 11 - A 63-year-old man presents with a 5-month history of non-specific back pain, fatigue...

    Incorrect

    • A 63-year-old man presents with a 5-month history of non-specific back pain, fatigue and loss of appetite. He has a medical history of type 2 diabetes mellitus controlled with diet, chronic obstructive pulmonary disease, and seasonal affective disorder. He has a 30 pack-year smoking history. On examination, his vital signs are within normal limits except for saturations of 94% on room air. A chest x-ray shows hyperinflated lung fields bilaterally, unchanged from a previous x-ray. Blood tests and urine analysis reveal a positive urinary Bence-Jones protein. Based on these findings, what is the most likely diagnosis?

      Your Answer:

      Correct Answer: Multiple myeloma

      Explanation:

      The diagnosis of multiple myeloma can be supported by the presence of Bence-Jones protein on urinary analysis, although it is not always necessary. This haematological malignancy of plasma cells is characterized by bone pain and lytic bone lesions. Hypercalcaemia can also indicate the presence of multiple myeloma.

      Ankylosing spondylitis is a chronic inflammatory arthritis that typically affects young men and is associated with HLA-B27. Symptoms include early-morning back pain that improves with exercise, and an elevated ESR may be observed.

      Chronic myeloid leukaemia (CML) is a haematological malignancy that is linked to genetic translocation on chromosome 9 (Philadelphia chromosome). It is characterized by high white cell count, splenomegaly, and blast cells seen on marrow biopsy.

      Gastric and pancreatic cancer may present with non-specific symptoms such as fatigue, weight loss, loss of appetite, and abdominal fullness or pain. Biochemistry may be normal or show raised inflammatory markers, and diagnosis is confirmed through biopsy following imaging.

      Understanding Multiple Myeloma: Features and Investigations

      Multiple myeloma is a type of cancer that affects the plasma cells in the bone marrow. It is most commonly found in patients aged 60-70 years. The disease is characterized by a range of symptoms, which can be remembered using the mnemonic CRABBI. These include hypercalcemia, renal damage, anemia, bleeding, bone lesions, and increased susceptibility to infection. Other features of multiple myeloma include amyloidosis, carpal tunnel syndrome, neuropathy, and hyperviscosity.

      To diagnose multiple myeloma, a range of investigations are required. Blood tests can reveal anemia, renal failure, and hypercalcemia. Protein electrophoresis can detect raised levels of monoclonal IgA/IgG proteins in the serum, while bone marrow aspiration can confirm the diagnosis if the number of plasma cells is significantly raised. Imaging studies, such as whole-body MRI or X-rays, can be used to detect osteolytic lesions.

      The diagnostic criteria for multiple myeloma require one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of the disease. Major criteria include the presence of plasmacytoma, 30% plasma cells in a bone marrow sample, or elevated levels of M protein in the blood or urine. Minor criteria include 10% to 30% plasma cells in a bone marrow sample, minor elevations in the level of M protein in the blood or urine, osteolytic lesions, or low levels of antibodies in the blood. Understanding the features and investigations of multiple myeloma is crucial for early detection and effective treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 12 - A 29-year-old man newly diagnosed with epilepsy visits his GP with complaints of...

    Incorrect

    • A 29-year-old man newly diagnosed with epilepsy visits his GP with complaints of fatigue. He has no significant medical history except for taking oral phenytoin and loratadine seasonally. He works as a construction site worker and has been experiencing a lot of stress at work due to the need to work extra shifts. During the consultation, he appears anxious and has a slim build. His blood test reveals macrocytic anaemia.

      What could be the probable reason for his symptoms and blood results?

      Your Answer:

      Correct Answer: Side effect of phenytoin

      Explanation:

      The most probable reason for the patient’s fatigue and abnormal blood results is the side effect of phenytoin. Phenytoin is an antifolate medication that can lead to folate deficiency, resulting in macrocytic anaemia, which is evident in the patient’s blood test. Fatigue is a common symptom of anaemia, which the patient has reported.

      Although lack of sleep may contribute to the patient’s tiredness, it alone cannot cause macrocytic anaemia.

      Hypothyroidism can cause macrocytic anaemia and lethargy, but it is less likely to be the cause of the patient’s symptoms. The patient has no history of thyroid disorders, and his slim build and anxiety are more typical of hyperthyroidism.

      Loratadine is a second-generation antihistamine that does not usually cause drowsiness.

      Understanding Macrocytic Anaemia

      Macrocytic anaemia is a type of anaemia that can be classified into two categories: megaloblastic and normoblastic. Megaloblastic anaemia is caused by a deficiency in vitamin B12 or folate, which leads to the production of abnormally large red blood cells in the bone marrow. This type of anaemia can also be caused by certain medications, alcohol, liver disease, hypothyroidism, pregnancy, and myelodysplasia.

      On the other hand, normoblastic anaemia is caused by an increase in the number of immature red blood cells, known as reticulocytes, in the bone marrow. This can occur as a result of certain medications, such as methotrexate, or in response to other underlying medical conditions.

      It is important to identify the underlying cause of macrocytic anaemia in order to provide appropriate treatment. This may involve addressing any nutritional deficiencies, managing underlying medical conditions, or adjusting medications. With proper management, most cases of macrocytic anaemia can be successfully treated.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 13 - A 67-year-old woman presents to haematology with fevers, tiredness, and unexplained weight loss....

    Incorrect

    • A 67-year-old woman presents to haematology with fevers, tiredness, and unexplained weight loss. She has painless cervical lymphadenopathy on examination. The haematologist suspects follicular lymphoma and orders a lymph node biopsy to confirm the diagnosis. Which translocation is expected to be detected through cytogenetics?

      Your Answer:

      Correct Answer: Translocation t(14;18)

      Explanation:

      Genetics of Haematological Malignancies

      Haematological malignancies are cancers that affect the blood, bone marrow, and lymphatic system. These cancers are often associated with specific genetic abnormalities, such as translocations. Here are some common translocations and their associated haematological malignancies:

      – Philadelphia chromosome (t(9;22)): This translocation is present in more than 95% of patients with chronic myeloid leukaemia (CML). It results in the fusion of the Abelson proto-oncogene with the BCR gene on chromosome 22, creating the BCR-ABL gene. This gene codes for a fusion protein with excessive tyrosine kinase activity, which is a poor prognostic indicator in acute lymphoblastic leukaemia (ALL).

      – t(15;17): This translocation is seen in acute promyelocytic leukaemia (M3) and involves the fusion of the PML and RAR-alpha genes.

      – t(8;14): Burkitt’s lymphoma is associated with this translocation, which involves the translocation of the MYC oncogene to an immunoglobulin gene.

      – t(11;14): Mantle cell lymphoma is associated with the deregulation of the cyclin D1 (BCL-1) gene.

      – t(14;18): Follicular lymphoma is associated with increased BCL-2 transcription due to this translocation.

      Understanding the genetic abnormalities associated with haematological malignancies is important for diagnosis, prognosis, and treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 14 - An 80-year-old woman visits her doctor complaining of excessive vaginal bleeding. After undergoing...

    Incorrect

    • An 80-year-old woman visits her doctor complaining of excessive vaginal bleeding. After undergoing an ultrasound scan and uterine biopsy, it is revealed that she has an endometrial tumor located in the uterine fundus. Which group of nearby lymph nodes will be the primary location for lymphatic metastasis of this tumor?

      Your Answer:

      Correct Answer: Para-aortic lymph nodes

      Explanation:

      The lymphatic drainage of the uterine fundus is similar to that of the ovaries, running alongside the ovarian vessels and draining into the para-aortic lymph nodes. Therefore, option 4 is correct. Options 1, 2, and 5 are incorrect as they refer to the drainage of the cervix and uterine body, which is different from that of the uterine fundus. Option 3 is also incorrect as the external iliac lymph nodes are not involved in the drainage of the uterine fundus.

      Lymphatic Drainage of Female Reproductive Organs

      The lymphatic drainage of the female reproductive organs is a complex system that involves multiple nodal stations. The ovaries drain to the para-aortic lymphatics via the gonadal vessels. The uterine fundus has a lymphatic drainage that runs with the ovarian vessels and may thus drain to the para-aortic nodes. Some drainage may also pass along the round ligament to the inguinal nodes. The body of the uterus drains through lymphatics contained within the broad ligament to the iliac lymph nodes. The cervix drains into three potential nodal stations; laterally through the broad ligament to the external iliac nodes, along the lymphatics of the uterosacral fold to the presacral nodes and posterolaterally along lymphatics lying alongside the uterine vessels to the internal iliac nodes. Understanding the lymphatic drainage of the female reproductive organs is important for the diagnosis and treatment of gynecological cancers.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 15 - A 70-year-old man is undergoing investigation for small intestine cancer due to his...

    Incorrect

    • A 70-year-old man is undergoing investigation for small intestine cancer due to his history of Crohn's disease. An adenocarcinoma of his duodenum is detected through endoscopy and histology. The oncologist is now examining his previous abdominal CT scan to determine if there is any nodal involvement.

      Which group of lymph nodes could potentially be affected in this scenario?

      Your Answer:

      Correct Answer: Superior mesenteric lymph nodes

      Explanation:

      The superior mesenteric lymph nodes are responsible for draining the duodenum, which is the second section of the gastrointestinal system. This lymphatic drainage is important for staging gastrointestinal cancers, and is similar to the blood supply of the gut. While the coeliac lymph nodes drain the first part of the gastrointestinal system, the inferior mesenteric lymph nodes drain the third part, and the internal iliac lymph nodes drain the lower part of the rectum and some of the anal canal. The para-aortic lymph nodes are not involved in the drainage of the gastrointestinal system, but instead drain the genito-urinary system. It is important to understand the correct lymphatic drainage patterns for accurate cancer staging.

      Lymphatic drainage is the process by which lymphatic vessels carry lymph, a clear fluid containing white blood cells, away from tissues and organs and towards lymph nodes. The lymphatic vessels that drain the skin and follow venous drainage are called superficial lymphatic vessels, while those that drain internal organs and structures follow the arteries and are called deep lymphatic vessels. These vessels eventually lead to lymph nodes, which filter and remove harmful substances from the lymph before it is returned to the bloodstream.

      The lymphatic system is divided into two main ducts: the right lymphatic duct and the thoracic duct. The right lymphatic duct drains the right side of the head and right arm, while the thoracic duct drains everything else. Both ducts eventually drain into the venous system.

      Different areas of the body have specific primary lymph node drainage sites. For example, the superficial inguinal lymph nodes drain the anal canal below the pectinate line, perineum, skin of the thigh, penis, scrotum, and vagina. The deep inguinal lymph nodes drain the glans penis, while the para-aortic lymph nodes drain the testes, ovaries, kidney, and adrenal gland. The axillary lymph nodes drain the lateral breast and upper limb, while the internal iliac lymph nodes drain the anal canal above the pectinate line, lower part of the rectum, and pelvic structures including the cervix and inferior part of the uterus. The superior mesenteric lymph nodes drain the duodenum and jejunum, while the inferior mesenteric lymph nodes drain the descending colon, sigmoid colon, and upper part of the rectum. Finally, the coeliac lymph nodes drain the stomach.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 16 - A 25-year-old male is getting a routine check-up from his family doctor before...

    Incorrect

    • A 25-year-old male is getting a routine check-up from his family doctor before starting a new workout regimen at the gym. He has a clean medical history and does not smoke or drink. He is currently pursuing a graduate degree in political science. The doctor orders a CBC and other tests.

      The patient returns to the doctor's office a week later for the test results. The CBC shows that his platelet count is low. However, he does not have any signs of bleeding from his nose or mouth, and there are no rashes on his skin.

      The doctor suspects that this may be due to platelet in vitro agglutination.

      What could have caused this condition?

      Your Answer:

      Correct Answer: Ethylenediaminetetraacetic acid (EDTA)

      Explanation:

      EDTA is known to induce pseudothrombocytopenia, which is a condition where platelet counts are falsely reported as low due to EDTA-dependent platelet aggregation. On the other hand, sodium fluoride inhibits glycolysis and prevents enzymes from functioning, leading to the depletion of substrates like glucose during storage. While sodium citrate, sodium oxalate, and lithium heparin are all anticoagulants commonly found in vacutainers, they are not linked to thrombocytopenia.

      Causes of Thrombocytopenia

      Thrombocytopenia is a medical condition characterized by a low platelet count in the blood. The severity of thrombocytopenia can vary, with some cases being more severe than others. Severe thrombocytopenia can be caused by conditions such as immune thrombocytopenia (ITP), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), and haematological malignancy. On the other hand, moderate thrombocytopenia can be caused by heparin-induced thrombocytopenia (HIT), drug-induced factors such as quinine, diuretics, sulphonamides, aspirin, and thiazides, alcohol, liver disease, hypersplenism, viral infections such as EBV, HIV, and hepatitis, pregnancy, SLE/antiphospholipid syndrome, and vitamin B12 deficiency. It is important to note that pseudothrombocytopenia can also occur as a result of using EDTA as an anticoagulant.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 17 - Which of the following is the least probable cause of an extended prothrombin...

    Incorrect

    • Which of the following is the least probable cause of an extended prothrombin time?

      Your Answer:

      Correct Answer: Acquired factor 12 deficiency

      Explanation:

      Cholestatic jaundice and prolonged antibiotic therapy can lead to a deficiency in vitamin K.

      Abnormal coagulation can be caused by various factors such as heparin, warfarin, disseminated intravascular coagulation (DIC), and liver disease. Heparin prevents the activation of factors 2, 9, 10, and 11, while warfarin affects the synthesis of factors 2, 7, 9, and 10. DIC affects factors 1, 2, 5, 8, and 11, and liver disease affects factors 1, 2, 5, 7, 9, 10, and 11.

      When interpreting blood clotting test results, different disorders can be identified based on the levels of activated partial thromboplastin time (APTT), prothrombin time (PT), and bleeding time. Haemophilia is characterized by increased APTT levels, normal PT levels, and normal bleeding time. On the other hand, von Willebrand’s disease is characterized by increased APTT levels, normal PT levels, and increased bleeding time. Lastly, vitamin K deficiency is characterized by increased APTT and PT levels, and normal bleeding time. Proper interpretation of these results is crucial in diagnosing and treating coagulation disorders.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 18 - A 76-year-old male is undergoing treatment for pancytopenia with an unknown cause. His...

    Incorrect

    • A 76-year-old male is undergoing treatment for pancytopenia with an unknown cause. His recent blood test revealed a decreased platelet count.

      What are the typical factors that stimulate the production of platelets?

      Your Answer:

      Correct Answer: Thrombopoietin

      Explanation:

      Platelets, also known as thrombocytes, are derived from myeloid stem cells, similar to red blood cells. The process involves the development of a megakaryocyte from a common myeloid progenitor cell. Megakaryocytes are large cells with multilobulated nuclei that grow to become massive before breaking up to form platelets.

      The primary signal responsible for megakaryocyte and platelet production is thrombopoietin.

      Erythropoietin initiates the signal for red blood cell production, while granulocyte-colony stimulating factor stimulates the bone marrow to produce granulocytes. Interleukin-5 is a cytokine that stimulates the proliferation and activation of eosinophils.

      Haematopoiesis: The Generation of Immune Cells

      Haematopoiesis is the process by which immune cells are produced from haematopoietic stem cells in the bone marrow. These stem cells give rise to two main types of progenitor cells: myeloid and lymphoid progenitor cells. All immune cells are derived from these progenitor cells.

      The myeloid progenitor cells generate cells such as macrophages/monocytes, dendritic cells, neutrophils, eosinophils, basophils, and mast cells. On the other hand, lymphoid progenitor cells give rise to T cells, NK cells, B cells, and dendritic cells.

      This process is essential for the proper functioning of the immune system. Without haematopoiesis, the body would not be able to produce the necessary immune cells to fight off infections and diseases. Understanding haematopoiesis is crucial in developing treatments for diseases that affect the immune system.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 19 - As a medical student working on a medical ward, you have a patient...

    Incorrect

    • As a medical student working on a medical ward, you have a patient who is 12 hours post a blood transfusion. The patient has developed a new cough and difficulty breathing, and their observations show new hypotension and a fever. What is the probable cause of these symptoms?

      Your Answer:

      Correct Answer: Transfusion-related acute lung injury

      Explanation:

      Transfusion reactions can be classified as immunological or non-immunological. Immunological reactions are caused by anti-HLA or other antibodies in the donor blood, while non-immunological reactions are triggered by an inflammatory cascade with lipids found in blood products.

      Symptoms of transfusion-related acute lung injury (TRALI) include dyspnea, cough, fever, and hypotension. Signs and investigations may reveal hypoxemia and pulmonary infiltrates visible on a chest x-ray.

      Fluid overload, on the other hand, typically presents with dyspnea, orthopnea, and paroxysmal nocturnal dyspnea.

      Severe allergic reactions are rare but may occur when the immune system attacks the donated blood, usually due to a mismatch in blood type. Symptoms may include urticaria, edema, dizziness, and headaches.

      Blood product transfusion complications can be categorized into immunological, infective, and other complications. Immunological complications include acute haemolytic reactions, non-haemolytic febrile reactions, and allergic/anaphylaxis reactions. Infective complications may arise due to transmission of vCJD, although measures have been taken to minimize this risk. Other complications include transfusion-related acute lung injury (TRALI), transfusion-associated circulatory overload (TACO), hyperkalaemia, iron overload, and clotting.

      Non-haemolytic febrile reactions are thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage. These reactions may occur in 1-2% of red cell transfusions and 10-30% of platelet transfusions. Minor allergic reactions may also occur due to foreign plasma proteins, while anaphylaxis may be caused by patients with IgA deficiency who have anti-IgA antibodies.

      Acute haemolytic transfusion reaction is a serious complication that results from a mismatch of blood group (ABO) which causes massive intravascular haemolysis. Symptoms begin minutes after the transfusion is started and include a fever, abdominal and chest pain, agitation, and hypotension. Treatment should include immediate transfusion termination, generous fluid resuscitation with saline solution, and informing the lab. Complications include disseminated intravascular coagulation and renal failure.

      TRALI is a rare but potentially fatal complication of blood transfusion that is characterized by the development of hypoxaemia/acute respiratory distress syndrome within 6 hours of transfusion. On the other hand, TACO is a relatively common reaction due to fluid overload resulting in pulmonary oedema. As well as features of pulmonary oedema, the patient may also be hypertensive, a key difference from patients with TRALI.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 20 - A 35-year-old man is diagnosed with a DVT in his right leg, which...

    Incorrect

    • A 35-year-old man is diagnosed with a DVT in his right leg, which is determined to be caused by a genetic disorder. What is the most prevalent hereditary factor leading to DVT?

      Your Answer:

      Correct Answer: Factor V Leiden

      Explanation:

      Deep vein thrombosis is a condition that occurs more frequently in Caucasians than in people of black African, Far East Asian, native Australian, and native American origin. The most common heritable causes of DVT, in descending order, are Factor V Leiden, Prothrombin G20210A variant, Protein C deficiency, Protein S deficiency, and Antithrombin deficiency. However, Von Willebrand disease and thalassaemia are not associated with DVT.

      Understanding Factor V Leiden

      Factor V Leiden is a common inherited thrombophilia, affecting around 5% of the UK population. It is caused by a mutation in the Factor V Leiden protein, resulting in activated factor V being inactivated 10 times more slowly by activated protein C than normal. This leads to activated protein C resistance, which increases the risk of venous thrombosis. Heterozygotes have a 4-5 fold risk of venous thrombosis, while homozygotes have a 10 fold risk, although the prevalence of homozygotes is much lower at 0.05%.

      Despite its prevalence, screening for Factor V Leiden is not recommended, even after a venous thromboembolism. This is because a previous thromboembolism itself is a risk factor for further events, and specific management should be based on this rather than the particular thrombophilia identified.

      Other inherited thrombophilias include Prothrombin gene mutation, Protein C deficiency, Protein S deficiency, and Antithrombin III deficiency. The table below shows the prevalence and relative risk of venous thromboembolism for each of these conditions.

      Overall, understanding Factor V Leiden and other inherited thrombophilias can help healthcare professionals identify individuals at higher risk of venous thrombosis and provide appropriate management to prevent future events.

      Condition | Prevalence | Relative risk of VTE
      — | — | —
      Factor V Leiden (heterozygous) | 5% | 4
      Factor V Leiden (homozygous) | 0.05% | 10
      Prothrombin gene mutation (heterozygous) | 1.5% | 3
      Protein C deficiency | 0.3% | 10
      Protein S deficiency | 0.1% | 5-10
      Antithrombin III deficiency | 0.02% | 10-20

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 21 - A mother brings her 6-month-old baby boy to your general practice, as he...

    Incorrect

    • A mother brings her 6-month-old baby boy to your general practice, as he was diagnosed with DiGeorge syndrome at birth. She was informed that one of the consequences of this condition is the failure of thymus development, which can impact her baby's blood cells. Specifically, which type of blood cell will be affected?

      Your Answer:

      Correct Answer: T lymphocytes

      Explanation:

      The correct answer is T lymphocytes, as the thymus plays a role in their maturation. DiGeorge syndrome is caused by a microdeletion on chromosome 22, resulting in the failure of development of the third and fourth pharyngeal arches. The syndrome is characterized by cardiac abnormalities, abnormal facies, thymus aplasia, cleft palate, and hypoparathyroidism, which can be remembered with the acronym CATCH.

      The Thymus Gland: Development, Structure, and Function

      The thymus gland is an encapsulated organ that develops from the third and fourth pharyngeal pouches. It descends to the anterior superior mediastinum and is subdivided into lobules, each consisting of a cortex and a medulla. The cortex is made up of tightly packed lymphocytes, while the medulla is mostly composed of epithelial cells. Hassall’s corpuscles, which are concentrically arranged medullary epithelial cells that may surround a keratinized center, are also present.

      The inferior parathyroid glands, which also develop from the third pharyngeal pouch, may be located with the thymus gland. The thymus gland’s arterial supply comes from the internal mammary artery or pericardiophrenic arteries, while its venous drainage is to the left brachiocephalic vein. The thymus gland plays a crucial role in the development and maturation of T-cells, which are essential for the immune system’s proper functioning.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 22 - A 5-year-old girl is brought to the emergency department by her parents after...

    Incorrect

    • A 5-year-old girl is brought to the emergency department by her parents after falling off the monkey bars at the playground. Shortly after, her left elbow became swollen and very painful. Her parents are worried because her father has a history of von Willebrand disease. The patient is evaluated for a bleeding disorder. The girl has no previous medical issues and is generally healthy.

      What is the most probable blood test result for this patient?

      Your Answer:

      Correct Answer: Increased APTT, normal bleeding time

      Explanation:

      Haemophilia A is the most likely diagnosis for the child based on the family history and presentation of haemarthrosis. Haemophilia is a genetic condition that affects clotting factors VIII or IX, which are part of the intrinsic pathway of the clotting cascade. APTT is a test that measures the intrinsic and common clotting cascades, but it does not include factor IX, so haemophilia B may not always show an abnormal APTT. PT measures the extrinsic and common pathways of the clotting cascade and is associated with factors I, II, V, VII, and X. Bleeding time measures platelet function, which is normal in haemophilia. Therefore, APTT may be raised, PT will be normal, and bleeding time will be normal in haemophilia.

      Haemophilia is a genetic disorder that affects blood coagulation and is inherited in an X-linked recessive manner. It is possible for up to 30% of patients to have no family history of the condition. Haemophilia A is caused by a deficiency of factor VIII, while haemophilia B, also known as Christmas disease, is caused by a lack of factor IX.

      The symptoms of haemophilia include haemoarthroses, haematomas, and prolonged bleeding after surgery or trauma. Blood tests can reveal a prolonged APTT, while the bleeding time, thrombin time, and prothrombin time are normal. However, up to 10-15% of patients with haemophilia A may develop antibodies to factor VIII treatment.

      Overall, haemophilia is a serious condition that can cause significant bleeding and other complications. It is important for individuals with haemophilia to receive appropriate medical care and treatment to manage their symptoms and prevent further complications.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 23 - A 25-year-old female visits her GP complaining of weight loss, fatigue, and night...

    Incorrect

    • A 25-year-old female visits her GP complaining of weight loss, fatigue, and night sweats that have been ongoing for the past 2 months. During the examination, the GP discovers cervical and axillary lymphadenopathy and hepatosplenomegaly. The patient is referred to the hospital for further investigation, which includes a biopsy of her cervical lymph nodes.

      The biopsy report reveals the presence of Reed-Sternberg cells. These cells belong to the same lineage as which of the following cells?

      Your Answer:

      Correct Answer: NK cells

      Explanation:

      Common lymphoid progenitor cells give rise to NK cells, as well as B-cells and T-cells. The biopsy of the patient in this case reveals Reed-Sternberg cells, indicating Hodgkin’s lymphoma, a cancer of B-cells. Platelets, monocytes, basophils, and erythrocytes, on the other hand, are derived from common myeloid progenitor cells.

      Haematopoiesis: The Generation of Immune Cells

      Haematopoiesis is the process by which immune cells are produced from haematopoietic stem cells in the bone marrow. These stem cells give rise to two main types of progenitor cells: myeloid and lymphoid progenitor cells. All immune cells are derived from these progenitor cells.

      The myeloid progenitor cells generate cells such as macrophages/monocytes, dendritic cells, neutrophils, eosinophils, basophils, and mast cells. On the other hand, lymphoid progenitor cells give rise to T cells, NK cells, B cells, and dendritic cells.

      This process is essential for the proper functioning of the immune system. Without haematopoiesis, the body would not be able to produce the necessary immune cells to fight off infections and diseases. Understanding haematopoiesis is crucial in developing treatments for diseases that affect the immune system.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 24 - You are developing a research project to evaluate the impact of a novel...

    Incorrect

    • You are developing a research project to evaluate the impact of a novel anticoagulant on the coagulation cascade. Your focus is on the intrinsic pathway. What parameter will you measure?

      Your Answer:

      Correct Answer: aPTT

      Explanation:

      The aPTT time is the most effective way to evaluate the intrinsic pathway of the clotting cascade. If the aPTT time is prolonged, it may indicate haemophilia or the use of heparin.

      To assess the extrinsic pathway, the prothrombin time (PT) is the preferred measurement.

      The thrombin time is a test that evaluates the formation of fibrin from fibrinogen in plasma. It can be prolonged by heparin, fibrin degradation products, and fibrinogen deficiency.

      A 50:50 mixing study is utilized to determine whether a prolonged PT or aPTT is caused by a factor deficiency or a factor inhibitor.

      The Coagulation Cascade: Two Pathways to Fibrin Formation

      The coagulation cascade is a complex process that leads to the formation of a blood clot. There are two pathways that can lead to fibrin formation: the intrinsic pathway and the extrinsic pathway. The intrinsic pathway involves components that are already present in the blood and has a minor role in clotting. It is initiated by subendothelial damage, such as collagen, which leads to the formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and Factor 12. This complex activates Factor 11, which in turn activates Factor 9. Factor 9, along with its co-factor Factor 8a, forms the tenase complex, which activates Factor 10.

      The extrinsic pathway, on the other hand, requires tissue factor released by damaged tissue. This pathway is initiated by tissue damage, which leads to the binding of Factor 7 to tissue factor. This complex activates Factor 9, which works with Factor 8 to activate Factor 10. Both pathways converge at the common pathway, where activated Factor 10 causes the conversion of prothrombin to thrombin. Thrombin hydrolyses fibrinogen peptide bonds to form fibrin and also activates factor 8 to form links between fibrin molecules.

      Finally, fibrinolysis occurs, which is the process of clot resorption. Plasminogen is converted to plasmin to facilitate this process. It is important to note that certain factors are involved in both pathways, such as Factor 10, and that some factors are vitamin K dependent, such as Factors 2, 7, 9, and 10. The intrinsic pathway can be assessed by measuring the activated partial thromboplastin time (APTT), while the extrinsic pathway can be assessed by measuring the prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 25 - A 14-year-old girl with beta thalassaemia major is receiving counselling from her haematologist...

    Incorrect

    • A 14-year-old girl with beta thalassaemia major is receiving counselling from her haematologist regarding the potential complications of her condition. The doctor explains that frequent blood transfusions may result in iron overload, which can result in liver damage and heart failure. What is an example of an iron chelation medication?

      Your Answer:

      Correct Answer: Deferiprone

      Explanation:

      To prevent complications from iron overload caused by frequent transfusions in beta-thalassaemia major, iron chelation therapy is crucial. Iron chelation agents such as Deferiprone, Deferoxamine, and Deferasirox are commonly used for this purpose. Trientine is a copper chelator used in Wilson’s disease, while Dimercaptosuccinic acid is used as a lead chelator. Penicillamine is primarily used to treat copper toxicity.

      Understanding Beta-Thalassaemia Major

      Beta-thalassaemia major is a genetic disorder that results from the absence of beta globulin chains on chromosome 11. This condition typically presents in the first year of life with symptoms such as failure to thrive and hepatosplenomegaly. Microcytic anaemia is also a common feature, with raised levels of HbA2 and HbF, but absent HbA.

      Management of beta-thalassaemia major involves repeated transfusions, which can lead to iron overload and organ failure. Therefore, iron chelation therapy, such as desferrioxamine, is crucial to prevent complications. It is important to understand the features and management of this condition to provide appropriate care for affected individuals.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 26 - A 28-year-old woman visits her doctor with complaints of fatigue. Upon further inquiry,...

    Incorrect

    • A 28-year-old woman visits her doctor with complaints of fatigue. Upon further inquiry, the doctor learns that she has been experiencing heavy menstrual bleeding for the past 8 months. To investigate further, the doctor orders a complete blood count and iron level test. What is the typical lifespan of a red blood cell?

      Your Answer:

      Correct Answer: 120 days

      Explanation:

      The bone marrow in large bones is responsible for the production of human red blood cells through erythropoiesis. Stem cells undergo a 7-day development process to become red blood cells, which then circulate for around 120 days before being eliminated by the spleen. Eryptosis, or programmed red cell death, occurs at the same rate as production.

      However, certain diseases can increase the rate of eryptosis, resulting in a shorter lifespan for red blood cells. These diseases include haemolytic uraemic syndrome, sepsis, malaria, sickle cell disease, thalassaemia, iron deficiency, and Wilson’s disease.

      Iron deficiency anaemia is a prevalent condition worldwide, with preschool-age children being the most affected. The lack of iron in the body leads to a decrease in red blood cells and haemoglobin, resulting in anaemia. The primary causes of iron deficiency anaemia are excessive blood loss, inadequate dietary intake, poor intestinal absorption, and increased iron requirements. Menorrhagia is the most common cause of blood loss in pre-menopausal women, while gastrointestinal bleeding is the most common cause in men and postmenopausal women. Vegans and vegetarians are more likely to develop iron deficiency anaemia due to the lack of meat in their diet. Coeliac disease and other conditions affecting the small intestine can prevent sufficient iron absorption. Children and pregnant women have increased iron demands, and the latter may experience dilution due to an increase in plasma volume.

      The symptoms of iron deficiency anaemia include fatigue, shortness of breath on exertion, palpitations, pallor, nail changes, hair loss, atrophic glossitis, post-cricoid webs, and angular stomatitis. To diagnose iron deficiency anaemia, a full blood count, serum ferritin, total iron-binding capacity, transferrin, and blood film tests are performed. Endoscopy may be necessary to rule out malignancy, especially in males and postmenopausal females with unexplained iron-deficiency anaemia.

      The management of iron deficiency anaemia involves identifying and treating the underlying cause. Oral ferrous sulfate is commonly prescribed, and patients should continue taking iron supplements for three months after the iron deficiency has been corrected to replenish iron stores. Iron-rich foods such as dark-green leafy vegetables, meat, and iron-fortified bread can also help. It is crucial to exclude malignancy by taking an adequate history and appropriate investigations if warranted.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 27 - A 28-year-old man comes to the hospital after experiencing two instances of bright...

    Incorrect

    • A 28-year-old man comes to the hospital after experiencing two instances of bright red urine in the morning. He is extremely anxious and informs the doctor that he has never had such an occurrence before. The man has recently started working at an engineering firm and is preparing to get married in a few months. He has been feeling fatigued for the past few months but attributed it to his job, which requires him to travel to construction sites daily. He has no significant medical history except for an appendectomy during childhood. A blood test shows a hemoglobin concentration of 11.5 g/dL and a reticulocyte count of 14% of red blood cells. What is the most probable finding that will be reported after flow cytometry of a blood sample from this patient?

      Your Answer:

      Correct Answer: CD55 and CD59 negative cells

      Explanation:

      Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic form of intrinsic hemolytic anemia that can present with symptoms such as hematuria, anemia, and venous thrombosis. The classic triad of PNH includes hemolytic anemia, pancytopenia, and venous thrombosis. The gold standard test for PNH is flow cytometry for CD59 and CD55, which shows a deficiency of these proteins on red and white blood cells.

      A deficiency of C3 is a complement deficiency disorder that increases the risk of recurrent bacterial infections. While a deficiency of CD59 or CD55 may be present in this patient, PNH patients typically have a deficiency of both proteins. Terminal complement deficiency, indicated by a deficiency of complements forming the membrane attack membrane, confers a high risk of infection with Neisseria organisms. Eculizumab, a humanized monoclonal antibody, is approved for the treatment of PNH and works by inhibiting the terminal complement cascade.

      Understanding Paroxysmal Nocturnal Haemoglobinuria

      Paroxysmal nocturnal haemoglobinuria (PNH) is a condition that causes the breakdown of haematological cells, mainly intravascular haemolysis. It is believed to be caused by a lack of glycoprotein glycosyl-phosphatidylinositol (GPI), which acts as an anchor that attaches surface proteins to the cell membrane. This leads to the improper binding of complement-regulating surface proteins, such as decay-accelerating factor (DAF), to the cell membrane. As a result, patients with PNH are more prone to venous thrombosis.

      PNH can affect red blood cells, white blood cells, platelets, or stem cells, leading to pancytopenia. Patients may also experience haemoglobinuria, which is characterized by dark-coloured urine in the morning. Thrombosis, such as Budd-Chiari syndrome, is also a common feature of PNH. In some cases, patients may develop aplastic anaemia.

      To diagnose PNH, flow cytometry of blood is used to detect low levels of CD59 and CD55. This has replaced Ham’s test as the gold standard investigation for PNH. Ham’s test involves acid-induced haemolysis, which normal red cells would not undergo.

      Management of PNH involves blood product replacement, anticoagulation, and stem cell transplantation. Eculizumab, a monoclonal antibody directed against terminal protein C5, is currently being trialled and is showing promise in reducing intravascular haemolysis. Understanding PNH is crucial in managing this condition and improving patient outcomes.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 28 - A 4-year-old boy visits the doctor complaining of occasional vomiting. He appears to...

    Incorrect

    • A 4-year-old boy visits the doctor complaining of occasional vomiting. He appears to be unstable while walking and his mother reports that he frequently complains of headaches. What is the probable diagnosis?

      Your Answer:

      Correct Answer: Medulloblastoma

      Explanation:

      Diagnosis of a Posterior Fossa Tumor in a Young Girl

      This young girl is showing symptoms of a posterior fossa tumor, which affects the cerebellar function. Ataxia, slurred speech, and double vision are common symptoms of this type of tumor. Additionally, headaches and vomiting are signs of increased intracranial pressure. The most likely diagnosis for this young girl is medulloblastoma, which is the most frequent posterior fossa tumor in children.

      Craniopharyngioma is an anterior fossa tumor that arises from the floor of the pituitary, making it an unlikely diagnosis for this young girl. Acute myeloid leukemia is rare in children and has a low rate of CNS involvement, unlike acute lymphoblastic leukemia. Ataxia telangiectasia is a hereditary condition that causes degeneration of multiple spinal cord tracts, but it would not present with features of a space-occupying lesion. Becker’s muscular dystrophy is an X-linked condition that causes weakness in boys.

      In summary, this young girl’s symptoms suggest a posterior fossa tumor, with medulloblastoma being the most likely diagnosis. It is important to accurately diagnose and treat this condition to ensure the best possible outcome for the patient.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 29 - An 83-year-old man is urgently referred for a colonoscopy due to worsening constipation...

    Incorrect

    • An 83-year-old man is urgently referred for a colonoscopy due to worsening constipation and newly detected iron deficiency anaemia. A suspicious lesion is observed in the descending colon during the procedure, and a biopsy is performed. Following diagnosis of colonic adenocarcinoma, the patient is referred for a CT scan to determine the stage of the cancer. Which lymph node would the lesion drain into?

      Your Answer:

      Correct Answer: Inferior mesenteric lymph nodes

      Explanation:

      The inferior mesenteric lymph nodes are responsible for draining the descending colon, which is where the initial lesion was identified during colonoscopy. Understanding the lymphatic drainage pathway is crucial in cancer diagnosis and treatment, as it can help predict potential sites of metastasis.

      For instance, cancers affecting the stomach, such as gastric adenocarcinomas or gastrointestinal stromal tumors, would be drained by the coeliac lymph nodes. On the other hand, the internal iliac lymph nodes are responsible for draining the anal canal (above the pectinate line), the lower part of the rectum, and other pelvic structures like the cervix. Therefore, cancers originating from these areas, such as squamous cell carcinoma of the cervix, would spread through these nodes.

      Para-aortic lymph nodes, on the other hand, drain cancers arising from the testes, ovaries, kidneys, and adrenal glands. Examples of these cancers include germ cell tumors (ovaries and testes), renal cell carcinomas, and phaeochromocytomas.

      Finally, the superior mesenteric lymph nodes are responsible for draining lesions arising in the duodenum and jejunum, such as small bowel adenocarcinomas and carcinoid tumors.

      Lymphatic drainage is the process by which lymphatic vessels carry lymph, a clear fluid containing white blood cells, away from tissues and organs and towards lymph nodes. The lymphatic vessels that drain the skin and follow venous drainage are called superficial lymphatic vessels, while those that drain internal organs and structures follow the arteries and are called deep lymphatic vessels. These vessels eventually lead to lymph nodes, which filter and remove harmful substances from the lymph before it is returned to the bloodstream.

      The lymphatic system is divided into two main ducts: the right lymphatic duct and the thoracic duct. The right lymphatic duct drains the right side of the head and right arm, while the thoracic duct drains everything else. Both ducts eventually drain into the venous system.

      Different areas of the body have specific primary lymph node drainage sites. For example, the superficial inguinal lymph nodes drain the anal canal below the pectinate line, perineum, skin of the thigh, penis, scrotum, and vagina. The deep inguinal lymph nodes drain the glans penis, while the para-aortic lymph nodes drain the testes, ovaries, kidney, and adrenal gland. The axillary lymph nodes drain the lateral breast and upper limb, while the internal iliac lymph nodes drain the anal canal above the pectinate line, lower part of the rectum, and pelvic structures including the cervix and inferior part of the uterus. The superior mesenteric lymph nodes drain the duodenum and jejunum, while the inferior mesenteric lymph nodes drain the descending colon, sigmoid colon, and upper part of the rectum. Finally, the coeliac lymph nodes drain the stomach.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 30 - A 50-year-old woman has recently been diagnosed with breast cancer and is now...

    Incorrect

    • A 50-year-old woman has recently been diagnosed with breast cancer and is now undergoing treatment with docetaxel. What is the mechanism of action for this particular treatment?

      Your Answer:

      Correct Answer: It prevents microtubule depolymerisation and disassembly, decreasing free tubulin

      Explanation:

      Docetaxel, a member of the taxane family, disrupts microtubule function by preventing depolymerisation and disassembly. This reduces free tubulin and halts cell division. Irinotecan inhibits topoisomerase I, preventing relaxation of supercoiled DNA, leading to DNA damage and cell death. Methotrexate inhibits dihydrofolate reductase and thymidylate synthesis, slowing and stopping DNA and protein synthesis necessary for normal cell cycle. Cisplatin binds to DNA, cross-linking and inhibiting replication. Doxorubicin stabilises the topoisomerase II complex, inhibiting DNA and RNA synthesis necessary for cell division.

      Cytotoxic agents are drugs that are used to kill cancer cells. There are several types of cytotoxic agents, each with their own mechanism of action and potential adverse effects. Alkylating agents, such as cyclophosphamide, work by causing cross-linking in DNA. However, they can also cause haemorrhagic cystitis, myelosuppression, and transitional cell carcinoma. Cytotoxic antibiotics, like bleomycin and anthracyclines, degrade preformed DNA and stabilize DNA-topoisomerase II complex, respectively. However, they can also cause lung fibrosis and cardiomyopathy. Antimetabolites, such as methotrexate and fluorouracil, inhibit dihydrofolate reductase and thymidylate synthesis, respectively. However, they can also cause myelosuppression, mucositis, and liver or lung fibrosis. Drugs that act on microtubules, like vincristine and docetaxel, inhibit the formation of microtubules and prevent microtubule depolymerisation & disassembly, respectively. However, they can also cause peripheral neuropathy, myelosuppression, and paralytic ileus. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I, which prevents relaxation of supercoiled DNA. However, they can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin and hydroxyurea, cause cross-linking in DNA and inhibit ribonucleotide reductase, respectively. However, they can also cause ototoxicity, peripheral neuropathy, hypomagnesaemia, and myelosuppression.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 31 - A 35-year-old male is referred to the medical assessment unit by his physician...

    Incorrect

    • A 35-year-old male is referred to the medical assessment unit by his physician to investigate the possibility of thrombotic thrombocytopenic purpura (TTP) after presenting with a fever of 38.5ºC. His recent urea and electrolyte results indicate declining kidney function, with a creatinine level three times higher than his usual baseline. What is the pathophysiology underlying TTP?

      Your Answer:

      Correct Answer: Failure to cleave von Willebrand factor normally

      Explanation:

      The absence of a plasma protease responsible for breaking down ultra-large multimers of von Willebrand factor (vWF) is the cause of TTP. This results in the accumulation of unusually large vWF multimers in the plasma of TTP patients. It is important to note that autoimmune destruction of red blood cells is a different form of autoimmune hemolytic anaemia and is not related to TTP. Similarly, autoimmune destruction of platelets is seen in ITP, not TTP.

      Thrombotic Thrombocytopenic Purpura: Understanding its Pathogenesis, Features, and Causes

      Thrombotic thrombocytopenic purpura (TTP) is a rare condition that typically affects adult females. Its pathogenesis involves the abnormal formation of large and sticky multimers of von Willebrand’s factor, which causes platelets to clump within vessels. In TTP, there is a deficiency of ADAMTS13, a metalloprotease enzyme that breaks down these large multimers. This deficiency leads to the formation of microemboli, resulting in fluctuating neuro signs, microangiopathic haemolytic anaemia, thrombocytopenia, and renal failure. TTP overlaps with haemolytic uraemic syndrome (HUS).

      TTP can be caused by various factors, including post-infection (e.g., urinary, gastrointestinal), pregnancy, drugs (such as ciclosporin, oral contraceptive pill, penicillin, clopidogrel, and acyclovir), tumours, SLE, and HIV. It is essential to identify the underlying cause of TTP to provide appropriate treatment and prevent further complications.

      In summary, TTP is a rare condition that affects adult females and is caused by the abnormal formation of large and sticky multimers of von Willebrand’s factor. Its features include fluctuating neuro signs, microangiopathic haemolytic anaemia, thrombocytopenia, and renal failure. TTP can be caused by various factors, and identifying the underlying cause is crucial for proper treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 32 - A 62-year-old man visited his primary care physician after his family noticed a...

    Incorrect

    • A 62-year-old man visited his primary care physician after his family noticed a significant weight loss over the past few months. The man confirms the weight loss and denies intentionally trying to lose weight or any changes in his appetite. He also reports experiencing night sweats and a fever, which he attributes to a cold. The patient has a history of well-controlled hypertension and no surgical history. He has not traveled recently and does not smoke or drink alcohol. During the physical examination, an enlarged lymph node in the armpit and splenomegaly were noted. The most likely diagnosis was confirmed through fluorescent in-situ hybridization (FISH), which revealed a translocation of the heavy-chain immunoglobulin and cyclin D1. What is the most likely translocation found in this patient?

      Your Answer:

      Correct Answer: t(11;14)

      Explanation:

      Understanding Mantle Cell Lymphoma

      Mantle cell lymphoma is a type of B-cell lymphoma that is characterized by the over-expression of the cyclin D1 (BCL-1) gene due to a translocation (11;14). This cancer is identified by the presence of CD5+, CD19+, CD22+, and CD23- markers. Unfortunately, mantle cell lymphoma has a poor prognosis and is often associated with widespread lymphadenopathy.

      In summary, mantle cell lymphoma is a type of cancer that affects B-cells and is caused by a specific genetic translocation. It is identified by certain markers and is known for its poor prognosis and widespread lymphadenopathy. Understanding the basics of this disease can help with early detection and treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 33 - A 22-year-old male visits his doctor with cramp-like abdominal pain. He is healthy...

    Incorrect

    • A 22-year-old male visits his doctor with cramp-like abdominal pain. He is healthy and has no notable medical history. Nausea and vomiting are not reported. The doctor prescribes a medication for him to take home. Two days later, the patient returns to the doctor's office, reporting constipation, dry mouth, and difficulty urinating. Which medication is the most probable cause of these side effects?

      Your Answer:

      Correct Answer: Hyoscine butylbromide

      Explanation:

      Hyoscine bromide can cause constipation, dry mouth, and urinary retention as its adverse effects.

      When a patient in palliative care is unable to take oral medication due to various reasons such as nausea, dysphagia, intestinal obstruction, weakness or coma, a syringe driver should be considered. In the UK, there are two main types of syringe drivers: Graseby MS16A (blue) and Graseby MS26 (green). The delivery rate for the former is given in mm per hour, while the latter is given in mm per 24 hours.

      Most drugs are compatible with water for injection, but for certain drugs such as granisetron, ketamine, ketorolac, octreotide, and ondansetron, sodium chloride 0.9% is recommended. Commonly used drugs for various symptoms include cyclizine, levomepromazine, haloperidol, metoclopramide for nausea and vomiting, hyoscine hydrobromide, hyoscine butylbromide, or glycopyrronium bromide for respiratory secretions/bowel colic, midazolam, haloperidol, levomepromazine for agitation/restlessness, and diamorphine as the preferred opioid for pain.

      When mixing drugs, diamorphine is compatible with most other drugs used, including dexamethasone, haloperidol, hyoscine butylbromide, hyoscine hydrobromide, levomepromazine, metoclopramide, and midazolam. However, cyclizine may precipitate with diamorphine when given at higher doses, and it is incompatible with a number of drugs such as clonidine, dexamethasone, hyoscine butylbromide (occasional), ketamine, ketorolac, metoclopramide, midazolam, octreotide, and sodium chloride 0.9%.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 34 - As a medical student on a surgical team, the FY1 doctor requests that...

    Incorrect

    • As a medical student on a surgical team, the FY1 doctor requests that you conduct a group and save blood test for a patient prior to their operation. The patient, who is identified as being in their 50s, has blood group A and therefore has anti-B antibodies. What type of antibodies will they possess?

      Your Answer:

      Correct Answer: IgM

      Explanation:

      The IgM antibody is composed of five antibodies joined together and is primarily responsible for clumping antigens. Anti-A and anti-B antibodies are typically IgM and are produced during early childhood due to exposure to environmental factors like bacteria, viruses, and food.

      On the other hand, IgG is the most prevalent antibody and exists as a single antibody complex. IgD, on the other hand, is located on the surface of B-lymphocytes.

      Blood product transfusion complications can be categorized into immunological, infective, and other complications. Immunological complications include acute haemolytic reactions, non-haemolytic febrile reactions, and allergic/anaphylaxis reactions. Infective complications may arise due to transmission of vCJD, although measures have been taken to minimize this risk. Other complications include transfusion-related acute lung injury (TRALI), transfusion-associated circulatory overload (TACO), hyperkalaemia, iron overload, and clotting.

      Non-haemolytic febrile reactions are thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage. These reactions may occur in 1-2% of red cell transfusions and 10-30% of platelet transfusions. Minor allergic reactions may also occur due to foreign plasma proteins, while anaphylaxis may be caused by patients with IgA deficiency who have anti-IgA antibodies.

      Acute haemolytic transfusion reaction is a serious complication that results from a mismatch of blood group (ABO) which causes massive intravascular haemolysis. Symptoms begin minutes after the transfusion is started and include a fever, abdominal and chest pain, agitation, and hypotension. Treatment should include immediate transfusion termination, generous fluid resuscitation with saline solution, and informing the lab. Complications include disseminated intravascular coagulation and renal failure.

      TRALI is a rare but potentially fatal complication of blood transfusion that is characterized by the development of hypoxaemia/acute respiratory distress syndrome within 6 hours of transfusion. On the other hand, TACO is a relatively common reaction due to fluid overload resulting in pulmonary oedema. As well as features of pulmonary oedema, the patient may also be hypertensive, a key difference from patients with TRALI.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 35 - A 10-year-old girl comes to the doctor's office with purpura. She appears to...

    Incorrect

    • A 10-year-old girl comes to the doctor's office with purpura. She appears to be in good health, but her blood test reveals thrombocytopenia, lymphopenia, leukopenia, and anemia. What is the probable diagnosis?

      Your Answer:

      Correct Answer: Acute lymphoblastic leukaemia

      Explanation:

      Acute Lymphoblastic Leukaemia

      Acute lymphoblastic leukaemia (ALL) is a type of cancer that commonly affects children over the age of one. It occurs when a lymphocyte precursor, known as a ‘blast cell’, grows abnormally in the bone marrow, leading to a failure of normal blood cell production. This results in peripheral cytopenias, which can cause symptoms such as anaemia, recurrent infections, and purpura. While a raised peripheral white cell count may occur in severe or late-stage disease, it is not common.

      Compared to other types of leukaemia and lymphoma, ALL is more likely to present with bone marrow failure symptoms. Acute myeloid leukaemia, for example, is more common in the elderly and presents with a raised peripheral white cell count. Burkitt lymphoma, on the other hand, is a high-grade non-Hodgkin lymphoma that typically presents with lymphadenopathy. Chronic lymphocytic leukaemia is also more common in the elderly and presents with a peripheral lymphocytosis. Langerhans histiocytosis, a condition that affects antigen-presenting cells, is more common in young children and often affects the skin or bones. While it can cause marrow failure, it is a rare occurrence.

      In summary, ALL is a type of cancer that affects children and is caused by abnormal growth of blast cells in the bone marrow. It can cause symptoms of bone marrow failure, such as anaemia, recurrent infections, and purpura. While other types of leukaemia and lymphoma may present with different symptoms, ALL is more likely to present with bone marrow failure symptoms.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 36 - You are investigating the impact of HIV on T-cells during their maturation process....

    Incorrect

    • You are investigating the impact of HIV on T-cells during their maturation process. Which organ sample is necessary to meet the criteria of your research?

      Your Answer:

      Correct Answer: Thymus

      Explanation:

      The thymus is where T-cells undergo maturation, while they are produced in the bone marrow. Once mature, they can be found in the spleen and lymph nodes where they interact with antigen presenting cells. To investigate the impact of HIV on T-cell maturation, a thymus sample is necessary.

      Understanding the Lymphatic System

      The lymphatic system is composed of primary and secondary lymphatic organs, as well as lymph vessels. The primary lymphatic organs are the thymus and red bone marrow, which are responsible for the formation and maturation of lymphocytes. These organs contain pluripotent cells that give rise to immunocompetent B cells and pre-T cells. To become mature T cells, pre-T cells must migrate to the thymus.

      On the other hand, secondary lymphatic organs include lymph nodes, the spleen, tonsils, mucosa-associated lymphoid tissue (MALT), and Peyer’s patches. These organs filter lymphocytes and activate them to mount an immune response. Understanding the lymphatic system is crucial in comprehending how the body’s immune system works. By knowing the different organs and their functions, we can appreciate how the body fights off infections and diseases.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 37 - A 45-year-old woman has been found to carry a BRCA 1 mutation. Besides...

    Incorrect

    • A 45-year-old woman has been found to carry a BRCA 1 mutation. Besides breast cancer, what other type of cancer is she most susceptible to developing?

      Your Answer:

      Correct Answer: Ovarian cancer

      Explanation:

      It is more probable for individuals with a history of colorectal cancer to develop a second colorectal cancer. However, the risk of developing other types of cancer is only slightly elevated and does not warrant screening.

      Genetic Conditions and Their Association with Surgical Diseases

      Li-Fraumeni Syndrome is an autosomal dominant genetic condition caused by mutations in the p53 tumour suppressor gene. Individuals with this syndrome have a high incidence of malignancies, particularly sarcomas and leukaemias. The diagnosis is made when an individual develops sarcoma under the age of 45 or when a first-degree relative is diagnosed with any cancer below the age of 45 and another family member develops malignancy under the age of 45 or sarcoma at any age.

      BRCA 1 and 2 are genetic conditions carried on chromosome 17 and chromosome 13, respectively. These conditions are linked to developing breast cancer with a 60% risk and an associated risk of developing ovarian cancer with a 55% risk for BRCA 1 and 25% risk for BRCA 2. BRCA2 mutation is also associated with prostate cancer in men.

      Lynch Syndrome is another autosomal dominant genetic condition that causes individuals to develop colonic cancer and endometrial cancer at a young age. 80% of affected individuals will get colonic and/or endometrial cancer. High-risk individuals may be identified using the Amsterdam criteria, which include three or more family members with a confirmed diagnosis of colorectal cancer, two successive affected generations, and one or more colon cancers diagnosed under the age of 50 years.

      Gardners syndrome is an autosomal dominant familial colorectal polyposis that causes multiple colonic polyps. Extra colonic diseases include skull osteoma, thyroid cancer, and epidermoid cysts. Desmoid tumours are seen in 15% of individuals with this syndrome. Due to colonic polyps, most patients will undergo colectomy to reduce the risk of colorectal cancer. It is now considered a variant of familial adenomatous polyposis coli.

      Overall, these genetic conditions have a significant association with surgical diseases, and early identification and management can help reduce the risk of malignancies and other associated conditions.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 38 - A 67-year-old woman visits her general practitioner complaining of pelvic pain, weight loss,...

    Incorrect

    • A 67-year-old woman visits her general practitioner complaining of pelvic pain, weight loss, and vaginal bleeding that has persisted for 3 months. She has been menopausal for 15 years and is not currently taking any medication. Upon examination, no abnormalities are found in her abdomen or pelvis, and she is referred to a gynaecologist for urgent evaluation. Unfortunately, the patient is diagnosed with endometrial cancer that has spread to the fundus of her uterus.

      Which lymph node region is most likely to be affected by metastatic spread in this patient?

      Your Answer:

      Correct Answer: Para-aortic nodes

      Explanation:

      The para-aortic lymph nodes are responsible for draining the uterine fundus. This is because the ovaries develop in the abdomen and move down the posterior abdominal wall during fetal development, and their lymphatic drainage comes from the para-aortic nodes. Therefore, lymphatic spread is most likely to occur in this location.

      The inferior mesenteric nodes are not responsible for draining the uterine fundus, as they primarily drain hindgut structures from the transverse colon down to the rectum.

      Similarly, the internal iliac nodes are not responsible for draining the uterine fundus, as they primarily drain the inferior portion of the rectum, the anal canal above the pectinate line, and the pelvic viscera.

      The posterior mediastinal chain is also not responsible for draining the uterine fundus, as it primarily drains the oesophagus, mediastinum, and posterior surface of the diaphragm.

      Lymphatic Drainage of Female Reproductive Organs

      The lymphatic drainage of the female reproductive organs is a complex system that involves multiple nodal stations. The ovaries drain to the para-aortic lymphatics via the gonadal vessels. The uterine fundus has a lymphatic drainage that runs with the ovarian vessels and may thus drain to the para-aortic nodes. Some drainage may also pass along the round ligament to the inguinal nodes. The body of the uterus drains through lymphatics contained within the broad ligament to the iliac lymph nodes. The cervix drains into three potential nodal stations; laterally through the broad ligament to the external iliac nodes, along the lymphatics of the uterosacral fold to the presacral nodes and posterolaterally along lymphatics lying alongside the uterine vessels to the internal iliac nodes. Understanding the lymphatic drainage of the female reproductive organs is important for the diagnosis and treatment of gynecological cancers.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 39 - A 20-year-old male who migrated from Ghana during childhood presents with an intermittent...

    Incorrect

    • A 20-year-old male who migrated from Ghana during childhood presents with an intermittent painful morning erection that has lasted for the past 4 hours. He has never experienced this problem before and is typically healthy. On examination, he has mild splenomegaly. Laboratory investigations reveal:

      - Hemoglobin (Hb) level of 115 g/L (normal range for males: 135-180 g/L; females: 115-160 g/L)
      - Mean corpuscular volume (MCV) of 76 fL (normal range: 80-95 fL)

      The peripheral blood film shows multiple small red blood cells, a few sickle cells, and target cells. Based on these findings, what is the most probable genotype for his condition?

      Your Answer:

      Correct Answer: HbSC

      Explanation:

      Hb SC is a less severe variant of sickle cell disease that can be detected early through screening of children in the UK. This condition is characterized by the presence of both the sickle mutation and the HbC mutation, which results in a lysine substitution for glutamic acid on position 6 of the beta chain. While HbSC shares similarities with sickle cell disease, its symptoms are less frequent and severe. The severity of the disease can vary depending on the specific genotype, with HbAA being normal, HbAS being asymptomatic, HbSC/Sβ+ being moderately affected, and HbSS/Sβ0 being severely affected due to the absence of normal haemoglobin.

      Understanding Sickle-Cell Anaemia

      Sickle-cell anaemia is a genetic disorder that occurs when an abnormal haemoglobin chain, known as HbS, is synthesized due to an autosomal recessive condition. This condition is more common in people of African descent, as the heterozygous condition offers some protection against malaria. In the UK, around 10% of Afro-Caribbean individuals are carriers of HbS. Symptoms in homozygotes typically do not develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.

      The pathophysiology of sickle-cell anaemia involves the substitution of the polar amino acid glutamate with the non-polar valine in each of the two beta chains (codon 6) of haemoglobin. This substitution decreases the water solubility of deoxy-Hb, causing HbS molecules to polymerize and sickle in the deoxygenated state. HbAS patients sickle at p02 2.5 – 4 kPa, while HbSS patients sickle at p02 5 – 6 kPa. Sickle cells are fragile and can cause haemolysis, block small blood vessels, and lead to infarction.

      To diagnose sickle-cell anaemia, haemoglobin electrophoresis is the definitive test. It is essential to understand the pathophysiology and symptoms of sickle-cell anaemia to provide appropriate care and management for affected individuals.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 40 - A 30-year-old pregnant woman (28 weeks gestation) presents with severe abdominal pain and...

    Incorrect

    • A 30-year-old pregnant woman (28 weeks gestation) presents with severe abdominal pain and per-vaginal (PV) bleeding. Upon examination, it is suspected that she is experiencing placental abruption. While attempting to establish IV access, the patient suddenly develops epistaxis, bruising on her arms, and bleeding from the cannulation site. Blood test results reveal thrombocytopenia and low fibrinogen levels, and her prothrombin time (PT), activated partial thromboplastin time (APTT), and D-dimer results are all elevated. What is the most likely explanation for these complications?

      Your Answer:

      Correct Answer: Disseminated intravascular coagulopathy (DIC)

      Explanation:

      DIC is often associated with pregnancy complications such as placental abruption and shock, as well as bleeding from multiple sites and abnormal blood test results. Placenta praevia is characterized by painless vaginal bleeding, but when combined with other haematological results and occurring in a pregnant woman, it may indicate DIC rather than ITP. TTP typically presents with jaundice, low platelets, fever, renal complications, and CNS signs, which are not evident in this case, and clotting test results do not support this diagnosis. While von Willebrand’s disease can cause spontaneous bleeding, the platelet count is usually normal.

      Disseminated Intravascular Coagulation: A Condition of Simultaneous Coagulation and Haemorrhage

      Disseminated intravascular coagulation (DIC) is a medical condition characterized by simultaneous coagulation and haemorrhage. It is caused by the initial formation of thrombi that consume clotting factors and platelets, ultimately leading to bleeding. DIC can be caused by various factors such as infection, malignancy, trauma, liver disease, and obstetric complications.

      Clinically, bleeding is usually the dominant feature of DIC, accompanied by bruising, ischaemia, and organ failure. Blood tests can reveal prolonged clotting times, thrombocytopenia, decreased fibrinogen, and increased fibrinogen degradation products. The treatment of DIC involves addressing the underlying cause and providing supportive management.

      In summary, DIC is a serious medical condition that requires prompt diagnosis and management. It is important to identify the underlying cause and provide appropriate treatment to prevent further complications. With proper care and management, patients with DIC can recover and regain their health.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 41 - A 70-year-old man has just undergone an emergency repair for a ruptured abdominal...

    Incorrect

    • A 70-year-old man has just undergone an emergency repair for a ruptured abdominal aortic aneurysm. Preoperatively, he was taking aspirin, clopidogrel, and warfarin. Intraoperatively, he received 5000 units of unfractionated heparin before the application of the aortic cross clamp. Upon admission to the critical care unit, his blood results are as follows:

      Full blood count
      Hb 8 g/dl
      Platelets 40 * 109/l
      WBC 7.1 * 109/l

      His fibrin degradation products are measured and found to be markedly elevated. What is the likely cause of these results?

      Your Answer:

      Correct Answer: Disseminated intravascular coagulation

      Explanation:

      DIC is the most probable diagnosis due to the presence of low platelet counts and elevated FDP in this scenario.

      Understanding Disseminated Intravascular Coagulation

      Under normal conditions, the coagulation and fibrinolysis processes work together to maintain hemostasis. However, in cases of disseminated intravascular coagulation (DIC), these processes become dysregulated, leading to widespread clotting and bleeding. One of the critical factors in the development of DIC is the release of tissue factor (TF), a glycoprotein found on the surface of various cell types. TF is normally not in contact with the circulation but is exposed after vascular damage or in response to cytokines and endotoxins. Once activated, TF triggers the extrinsic pathway of coagulation, leading to the activation of the intrinsic pathway and the formation of clots.

      DIC can be caused by various factors, including sepsis, trauma, obstetric complications, and malignancy. Diagnosis of DIC typically involves a blood test that shows decreased platelet count and fibrinogen levels, prolonged prothrombin time and activated partial thromboplastin time, and increased fibrinogen degradation products. Microangiopathic hemolytic anemia may also be present, leading to the formation of schistocytes.

      Overall, understanding the pathophysiology and diagnosis of DIC is crucial for prompt and effective management of this potentially life-threatening condition.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 42 - Which one of the following statements relating to abnormal coagulation is not true?...

    Incorrect

    • Which one of the following statements relating to abnormal coagulation is not true?

      Your Answer:

      Correct Answer: The prothrombin time is prolonged in Haemophilia A

      Explanation:

      Haemophilia A is characterized by prolonged APTT and reduced levels of factor 8:C, while bleeding time and PT remain normal. Cholestatic jaundice hinders the absorption of vitamin K, which is fat-soluble. Patients who undergo massive transfusions, equivalent to more than 10 units of blood or their entire blood volume, are at risk of thrombocytopenia, as well as deficiencies in factor 5 and 8.

      Abnormal coagulation can be caused by various factors such as heparin, warfarin, disseminated intravascular coagulation (DIC), and liver disease. Heparin prevents the activation of factors 2, 9, 10, and 11, while warfarin affects the synthesis of factors 2, 7, 9, and 10. DIC affects factors 1, 2, 5, 8, and 11, and liver disease affects factors 1, 2, 5, 7, 9, 10, and 11.

      When interpreting blood clotting test results, different disorders can be identified based on the levels of activated partial thromboplastin time (APTT), prothrombin time (PT), and bleeding time. Haemophilia is characterized by increased APTT levels, normal PT levels, and normal bleeding time. On the other hand, von Willebrand’s disease is characterized by increased APTT levels, normal PT levels, and increased bleeding time. Lastly, vitamin K deficiency is characterized by increased APTT and PT levels, and normal bleeding time. Proper interpretation of these results is crucial in diagnosing and treating coagulation disorders.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 43 - A 60-year-old male presents with fatigue, pallor and a tingling sensation in both...

    Incorrect

    • A 60-year-old male presents with fatigue, pallor and a tingling sensation in both hands. Screening blood tests reveal:

      Hb 110 g/l (115-160 g/l)
      MCV 112 fl (82-100 fl)
      B12 140 ng/l (200-900 ng/l)

      What is the most frequent reason for this patient's macrocytic anaemia?

      Your Answer:

      Correct Answer: Pernicious anaemia

      Explanation:

      The primary cause of vitamin B12 deficiency is pernicious anaemia. This condition occurs when the stomach lining is destroyed by autoimmune factors, leading to reduced production of intrinsic factor. Intrinsic factor is responsible for binding B12 in the gut, and without it, B12 absorption is impaired. This can result in a deficiency of vitamin B12 and macrocytic anaemia, as well as neurological symptoms due to damage to spinal cord myelination.

      While a strict vegan diet and alcoholism can also lead to B12 deficiency, they are not the most common causes.

      Microcytic sideroblastic anaemia, on the other hand, is caused by lead poisoning, which impairs haem production.

      Vitamin B12 is essential for the development of red blood cells and the maintenance of the nervous system. It is absorbed through the binding of intrinsic factor, which is secreted by parietal cells in the stomach, and actively absorbed in the terminal ileum. A deficiency in vitamin B12 can be caused by pernicious anaemia, post gastrectomy, a vegan or poor diet, disorders or surgery of the terminal ileum, Crohn’s disease, or metformin use.

      Symptoms of vitamin B12 deficiency include macrocytic anaemia, a sore tongue and mouth, neurological symptoms, and neuropsychiatric symptoms such as mood disturbances. The dorsal column is usually affected first, leading to joint position and vibration issues before distal paraesthesia.

      Management of vitamin B12 deficiency involves administering 1 mg of IM hydroxocobalamin three times a week for two weeks, followed by once every three months if there is no neurological involvement. If a patient is also deficient in folic acid, it is important to treat the B12 deficiency first to avoid subacute combined degeneration of the cord.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 44 - What characteristic could serve as a reliable indicator of prognosis for a patient...

    Incorrect

    • What characteristic could serve as a reliable indicator of prognosis for a patient who has recently been diagnosed with acute lymphoblastic leukemia (ALL)?

      Your Answer:

      Correct Answer:

      Explanation:

      Prognostic Factors in Acute Lymphoblastic Leukemia

      Younger patients with acute lymphoblastic leukemia (ALL) have a better prognosis than older patients. In fact, the cure rate in children is around 90%, while it is less than 40% in adults. Additionally, male patients tend to fare worse than females, and they require a longer maintenance dose of chemotherapy (3 years versus 2 years). Interestingly, the Philadelphia chromosome, which is an effective treatment target in chronic myeloid leukemia, is actually a poor prognostic marker in ALL. Finally, higher white cell counts are associated with adverse outcomes, particularly if the count exceeds 100 ×106/ml.

      Overall, these prognostic factors can help clinicians predict the likelihood of a successful outcome in patients with ALL. By taking these factors into account, healthcare providers can tailor treatment plans to each patient’s individual needs and improve their chances of a positive outcome.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 45 - Which of the following processes enables phagocytosis to occur? ...

    Incorrect

    • Which of the following processes enables phagocytosis to occur?

      Your Answer:

      Correct Answer: Opsonisation

      Explanation:

      Phagocytosis is facilitated by opsonisation, which involves coating the micro-organism with antibody, C3b, and specific acute phase proteins. This leads to an increase in phagocytic cell surface receptors on macrophages and neutrophils, which is mediated by pro-inflammatory cytokines. As a result, these cells are able to engulf the micro-organism.

      Phagocytosis: The Process of Cell Ingestion

      Phagocytosis is the process by which cells ingest foreign materials or pathogens. The first step in this process is opsonisation, where the organism is coated by an antibody. The second step is adhesion to the cell surface, followed by pseudopodial extension to form a phagocytic vacuole. Finally, lysosomes fuse with the vacuole and degrade its contents.

      Phagocytosis is an essential process for the immune system to fight off infections and diseases. It is a complex process that involves multiple steps, including opsonisation, adhesion, and pseudopodial extension. The end result is the degradation of the foreign material or pathogen by lysosomes. Understanding the process of phagocytosis is crucial for developing treatments for diseases that involve the immune system.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 46 - A 68-year-old woman is referred to haematology via the 2-week wait pathway due...

    Incorrect

    • A 68-year-old woman is referred to haematology via the 2-week wait pathway due to worsening thoracic back pain that started 3 weeks ago. There is no history of trauma. Upon examination, the pain is exacerbated by movement and occurs even at rest and during bedtime. Her blood test results show a Hb level of 97 g/L (female normal range: 115-160 g/L), platelets of 200 * 109/L (normal range: 150-400 * 109/L), and WBC count of 4.0 * 109/L (normal range: 4.0-11.0 * 109/L). Additionally, her calcium level is 2.9 mmol/L (normal range: 2.1-2.6 mmol/L), phosphate level is 1.2 mmol/L (normal range: 0.8-1.4 mmol/L), magnesium level is 0.8 mmol/L (normal range: 0.7-1.0 mmol/L), TSH level is 5.0 mU/L (normal range: 0.5-5.5 mU/L), and free thyroxine (T4) level is 16 pmol/L (normal range: 9.0-18 pmol/L). Based on the likely diagnosis, what is the underlying pathophysiology that causes hypercalcemia?

      Your Answer:

      Correct Answer: Increased osteoclast activity in response to cytokines

      Explanation:

      Increased osteoclast activity in response to cytokines released by myeloma cells is the primary cause of hypercalcaemia in multiple myeloma, which typically affects individuals aged 60-70 years and presents with bone pain or pathological fractures from osteolytic lesions. Hypercalcaemia in kidney failure is associated with hyperphosphataemia and does not cause bone pain. Elevated calcitriol levels are linked to granulomatous disorders like sarcoidosis and tuberculosis, which do not typically cause bone pain. Rebound hypercalcaemia occurs after rhabdomyolysis, which usually results from a fall and long lie. Although primary hyperparathyroidism is a common cause of hypercalcaemia and can lead to bone pain or pathological fractures, it is not associated with anaemia.

      Understanding Multiple Myeloma: Features and Investigations

      Multiple myeloma is a type of cancer that affects the plasma cells in the bone marrow. It is most commonly found in patients aged 60-70 years. The disease is characterized by a range of symptoms, which can be remembered using the mnemonic CRABBI. These include hypercalcemia, renal damage, anemia, bleeding, bone lesions, and increased susceptibility to infection. Other features of multiple myeloma include amyloidosis, carpal tunnel syndrome, neuropathy, and hyperviscosity.

      To diagnose multiple myeloma, a range of investigations are required. Blood tests can reveal anemia, renal failure, and hypercalcemia. Protein electrophoresis can detect raised levels of monoclonal IgA/IgG proteins in the serum, while bone marrow aspiration can confirm the diagnosis if the number of plasma cells is significantly raised. Imaging studies, such as whole-body MRI or X-rays, can be used to detect osteolytic lesions.

      The diagnostic criteria for multiple myeloma require one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of the disease. Major criteria include the presence of plasmacytoma, 30% plasma cells in a bone marrow sample, or elevated levels of M protein in the blood or urine. Minor criteria include 10% to 30% plasma cells in a bone marrow sample, minor elevations in the level of M protein in the blood or urine, osteolytic lesions, or low levels of antibodies in the blood. Understanding the features and investigations of multiple myeloma is crucial for early detection and effective treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 47 - A 50-year-old female patient complains of feeling weak, exhausted, and fatigued. Upon examination,...

    Incorrect

    • A 50-year-old female patient complains of feeling weak, exhausted, and fatigued. Upon examination, her full blood count indicates acute lymphoblastic leukemia (ALL). Among the following options, which is linked to the poorest prognosis in ALL?

      Your Answer:

      Correct Answer: Age

      Explanation:

      Factors Associated with Positive Long-Term Outcome in Leukemia Patients

      Leukemia patients who are younger than 30 years old, have a white blood cell count of less than 30 ×109/L, and have a mediastinal mass are more likely to have a favorable long-term outcome. Additionally, those with a T cell or TMy immunophenotype and do not have the Philadelphia (Ph) chromosome also have a better prognosis. These clinical and biologic features are important factors to consider when assessing the potential outcome for leukemia patients. Proper identification and monitoring of these factors can aid in the development of effective treatment plans and improve patient outcomes.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 48 - A 29-year-old woman is a few minutes into receiving a blood transfusion after...

    Incorrect

    • A 29-year-old woman is a few minutes into receiving a blood transfusion after experiencing a postpartum haemorrhage when she experiences intense chest and abdominal pain. Her temperature has risen to 38.1ºC, and her BP is 80/60 mmHg. The medical team suspects that she is having an acute reaction to the blood product transfusion.

      What is the underlying mechanism of the probable transfusion reaction?

      Your Answer:

      Correct Answer: Host IgM antibody destruction of ABO-incompatible red blood cells (RBCs)

      Explanation:

      The correct mechanism of acute haemolytic transfusion reactions is the destruction of ABO-incompatible red blood cells (RBCs) by host IgM antibodies. These reactions typically occur due to human error in giving patients ABO-incompatible blood products. Symptoms include hypotension, fever, and abdominal and/or chest pain.

      Fluid overload, host anti-IgA antibodies reacting against donor IgA, and host antibodies reacting with donor white cell fragments are all incorrect mechanisms for acute haemolytic transfusion reactions. These mechanisms are associated with transfusion-associated circulatory overload (TACO), anaphylaxis to blood products in patients with IgA deficiency, and non-haemolytic febrile reactions, respectively. These conditions present with different symptoms and are not associated with the rapid onset of hypotension and abdominal pain seen in acute haemolytic transfusion reactions.

      Blood product transfusion complications can be categorized into immunological, infective, and other complications. Immunological complications include acute haemolytic reactions, non-haemolytic febrile reactions, and allergic/anaphylaxis reactions. Infective complications may arise due to transmission of vCJD, although measures have been taken to minimize this risk. Other complications include transfusion-related acute lung injury (TRALI), transfusion-associated circulatory overload (TACO), hyperkalaemia, iron overload, and clotting.

      Non-haemolytic febrile reactions are thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage. These reactions may occur in 1-2% of red cell transfusions and 10-30% of platelet transfusions. Minor allergic reactions may also occur due to foreign plasma proteins, while anaphylaxis may be caused by patients with IgA deficiency who have anti-IgA antibodies.

      Acute haemolytic transfusion reaction is a serious complication that results from a mismatch of blood group (ABO) which causes massive intravascular haemolysis. Symptoms begin minutes after the transfusion is started and include a fever, abdominal and chest pain, agitation, and hypotension. Treatment should include immediate transfusion termination, generous fluid resuscitation with saline solution, and informing the lab. Complications include disseminated intravascular coagulation and renal failure.

      TRALI is a rare but potentially fatal complication of blood transfusion that is characterized by the development of hypoxaemia/acute respiratory distress syndrome within 6 hours of transfusion. On the other hand, TACO is a relatively common reaction due to fluid overload resulting in pulmonary oedema. As well as features of pulmonary oedema, the patient may also be hypertensive, a key difference from patients with TRALI.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 49 - A 48-year-old woman presents to the clinic with complaints of abdominal pain and...

    Incorrect

    • A 48-year-old woman presents to the clinic with complaints of abdominal pain and constipation. During the examination, you observe blue lines on the gum margin. She also reports experiencing weakness in her legs over the past few days. What is the probable diagnosis?

      Your Answer:

      Correct Answer: Lead poisoning

      Explanation:

      Lead poisoning is a condition that should be considered when a patient presents with abdominal pain and neurological symptoms, along with acute intermittent porphyria. This condition is caused by defective ferrochelatase and ALA dehydratase function. Symptoms of lead poisoning include abdominal pain, peripheral neuropathy (mainly motor), neuropsychiatric features, fatigue, constipation, and blue lines on the gum margin (which is rare in children and only present in 20% of adult patients).

      To diagnose lead poisoning, doctors typically measure the patient’s blood lead level, with levels greater than 10 mcg/dl considered significant. A full blood count may also be performed, which can reveal microcytic anemia and red cell abnormalities such as basophilic stippling and clover-leaf morphology. Additionally, raised serum and urine levels of delta aminolaevulinic acid may be seen, which can sometimes make it difficult to differentiate from acute intermittent porphyria. Urinary coproporphyrin is also increased, while urinary porphobilinogen and uroporphyrin levels are normal to slightly increased. In children, lead can accumulate in the metaphysis of the bones, although x-rays are not typically part of the standard work-up.

      Various chelating agents are currently used to manage lead poisoning, including dimercaptosuccinic acid (DMSA), D-penicillamine, EDTA, and dimercaprol. These agents work to remove the lead from the body and can help alleviate symptoms.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 50 - You are evaluating a 43-year-old female patient at the breast cancer clinic who...

    Incorrect

    • You are evaluating a 43-year-old female patient at the breast cancer clinic who is undergoing chemotherapy treatment after a mastectomy. One of the medications she is taking is doxorubicin. What is the mechanism of action of this drug?

      Your Answer:

      Correct Answer: Stabilises DNA-topoisomerase II complex, inhibits DNA & RNA synthesis

      Explanation:

      Doxorubicin is an anthracycline that works by stabilizing the DNA-topoisomerase II complex and inhibiting DNA and RNA synthesis. It is used to treat acute leukemias, Hodgkin’s and non-Hodgkin’s lymphoma, and some solid tumors such as breast and sarcoma. However, it can cause cardiomyopathy as a potential complication. Ondansetron is a 5-HT3 antagonist that is used to manage chemotherapy-induced nausea and vomiting. Beta-blockers like bisoprolol and atenolol, on the other hand, inhibit beta-1 receptors and are used to treat hypertension, angina, heart failure, and atrial fibrillation. They are not cytotoxic medications. Cisplatin is a cytotoxic agent that inhibits cell division by causing cross-linking of DNA. It is used to treat various cancers such as testicular, lung, cervical, bladder, head and neck, and ovarian cancer. Methotrexate, another cytotoxic agent, inhibits dihydrofolate reductase and is commonly used to treat rheumatoid arthritis. However, it can cause gastrointestinal disturbance as a side effect.

      Cytotoxic agents are drugs that are used to kill cancer cells. There are several types of cytotoxic agents, each with their own mechanism of action and potential adverse effects. Alkylating agents, such as cyclophosphamide, work by causing cross-linking in DNA. However, they can also cause haemorrhagic cystitis, myelosuppression, and transitional cell carcinoma. Cytotoxic antibiotics, like bleomycin and anthracyclines, degrade preformed DNA and stabilize DNA-topoisomerase II complex, respectively. However, they can also cause lung fibrosis and cardiomyopathy. Antimetabolites, such as methotrexate and fluorouracil, inhibit dihydrofolate reductase and thymidylate synthesis, respectively. However, they can also cause myelosuppression, mucositis, and liver or lung fibrosis. Drugs that act on microtubules, like vincristine and docetaxel, inhibit the formation of microtubules and prevent microtubule depolymerisation & disassembly, respectively. However, they can also cause peripheral neuropathy, myelosuppression, and paralytic ileus. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I, which prevents relaxation of supercoiled DNA. However, they can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin and hydroxyurea, cause cross-linking in DNA and inhibit ribonucleotide reductase, respectively. However, they can also cause ototoxicity, peripheral neuropathy, hypomagnesaemia, and myelosuppression.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 51 - A 9-year-old girl is brought to the emergency department with acute onset pain...

    Incorrect

    • A 9-year-old girl is brought to the emergency department with acute onset pain in her hands for the past 2 hours. She has a history of recurrent infections. Physical examination shows tender diffuse swelling of her hands bilaterally.

      Her blood tests show:
      Hb 85 g/L Male: (119-150)
      Female: (119-150)
      Platelets 250 * 109/L (150 - 400)
      WBC 6 * 109/L (4.0 - 11.0)
      Mean corpuscular volume (MCV) 90 fL (80-100)

      Peripheral smear examination shows numerous sickled red blood cells (RBC) and Howell-jolly bodies. Haemoglobin electrophoresis confirms sickle cell disease.

      Which of the following is a beneficial prophylactic drug for her?

      Your Answer:

      Correct Answer: Hydroxyurea

      Explanation:

      Hydroxyurea is utilized in the prophylactic management of sickle cell anemia to prevent painful episodes by increasing the levels of HbF. The management of sickle cell disease involves two aspects: acute episodes and chronic management. Acute episodes are treated with adequate hydration and effective analgesia, while chronic management aims to prevent acute episodes and treat complications. Hydroxyurea has been proven to reduce the frequency of painful crises and the need for blood transfusions by increasing HbF levels, which has a higher affinity for oxygen than haemoglobin A. Acetaminophen is an analgesic that inhibits the cyclooxygenase enzyme and is only useful in mild pain cases. Methotrexate is a chemotherapeutic agent that has no role in sickle cell disease management.

      Managing Sickle-Cell Anaemia

      Sickle-cell anaemia is a genetic blood disorder that causes red blood cells to become misshapen and break down, leading to a range of complications. When a crisis occurs, management involves providing analgesia, rehydration, oxygen, and potentially antibiotics if there is evidence of infection. Blood transfusions may also be necessary, and in some cases, an exchange transfusion may be required if there are neurological complications.

      In the longer term, prophylactic management of sickle-cell anaemia involves the use of hydroxyurea, which increases the levels of HbF to prevent painful episodes. Additionally, it is recommended that sickle-cell patients receive the pneumococcal polysaccharide vaccine every five years to reduce the risk of infection. By implementing these management strategies, individuals with sickle-cell anaemia can better manage their condition and improve their quality of life.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 52 - A 22-year-old man presents to the physician with recurrent respiratory infections. He reports...

    Incorrect

    • A 22-year-old man presents to the physician with recurrent respiratory infections. He reports that his uncle had similar symptoms and passed away from a 'blood condition' three years ago. On physical examination, he has conjunctival pallor, a pulse of 110/min, and respirations of 19/min. Laboratory studies reveal a hemoglobin level of 100 g/L, platelets of 250 * 109/L, WBC of 7 * 109/L, a reticulocyte count of 6%, and an MCV of 84 fL. A peripheral smear shows numerous sickle-shaped red blood cells and Howell-jolly bodies. What additional investigation would be useful in confirming the diagnosis for this patient?

      Your Answer:

      Correct Answer: Hemoglobin electrophoresis

      Explanation:

      Sickle cell disease can be definitively diagnosed through haemoglobin electrophoresis. In the case of a patient experiencing an acute haemolytic episode due to sickle cell disease, normocytic anaemia with a high reticulocyte count and the presence of Howell jolly bodies indicate hyposplenism. A peripheral smear showing sickle cells is also highly indicative of sickle cell disease, which is an autosomal recessive condition that may be present in other family members.

      The osmotic fragility test is used to diagnose hereditary spherocytosis by exposing red blood cells to varying osmolarity and observing their fragility. Plasma folate deficiency can lead to macrocytic anaemia, but this is not the case in sickle cell disease. Flow cytometry is not useful in diagnosing sickle cell disease, but it can be used to classify leukemias and diagnose paroxysmal nocturnal haemoglobinuria.

      If an autoimmune cause is suspected, a Coombs test can be performed to confirm the pathogenesis, as in the case of haemolytic disease of the newborn. Haemoglobin electrophoresis is one of the definitive tests for diagnosing sickle cell trait and disease, as it shows a decrease in normal haemoglobin A and the presence of haemoglobin S. Genetic analysis can also confirm the diagnosis.

      Understanding Sickle-Cell Anaemia

      Sickle-cell anaemia is a genetic disorder that occurs when an abnormal haemoglobin chain, known as HbS, is synthesized due to an autosomal recessive condition. This condition is more common in people of African descent, as the heterozygous condition offers some protection against malaria. In the UK, around 10% of Afro-Caribbean individuals are carriers of HbS. Symptoms in homozygotes typically do not develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.

      The pathophysiology of sickle-cell anaemia involves the substitution of the polar amino acid glutamate with the non-polar valine in each of the two beta chains (codon 6) of haemoglobin. This substitution decreases the water solubility of deoxy-Hb, causing HbS molecules to polymerize and sickle in the deoxygenated state. HbAS patients sickle at p02 2.5 – 4 kPa, while HbSS patients sickle at p02 5 – 6 kPa. Sickle cells are fragile and can cause haemolysis, block small blood vessels, and lead to infarction.

      To diagnose sickle-cell anaemia, haemoglobin electrophoresis is the definitive test. It is essential to understand the pathophysiology and symptoms of sickle-cell anaemia to provide appropriate care and management for affected individuals.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 53 - A 27-year-old man with Von Willebrand's disease is scheduled for an intravenous infusion...

    Incorrect

    • A 27-year-old man with Von Willebrand's disease is scheduled for an intravenous infusion of desmopressin acetate. The medication works by triggering the release of von Willebrand factor from cells, which enhances factor VIII and the creation of the platelet plug in clotting. What substance is responsible for maintaining blood solubility and preventing platelet activation in individuals without clotting disorders?

      Your Answer:

      Correct Answer: Prostacyclin

      Explanation:

      Understanding the coagulation cascade is crucial, but it’s also important to know the substances that the body secretes to maintain normal blood vessel function and prevent excessive clotting. In primary haemostasis, the formation of a platelet plug is a critical step, and several substances in the blood vessels work against platelet activation to keep the blood flowing smoothly.

      Prostacyclin, which is produced from arachidonic acid, inhibits platelet activation. Nitric oxide prevents platelet adhesion to the vessel wall and also dilates blood vessels to increase blood flow. Endothelial ADPase inhibits ADP, which is a platelet activator.

      Fibrinogen, a large and soluble compound, is the precursor to fibrin, which forms an insoluble mesh to trap blood cells and platelets within a clot. This is the final step of the coagulation cascade, and the clot is further strengthened by fibrin-stabilising factor. Thromboxane, produced by activated platelets, increases platelet activation and constricts blood vessels, making it another thrombotic agent. Aggregated platelets produce ADP, which further enhances platelet aggregation.

      The Coagulation Cascade: Two Pathways to Fibrin Formation

      The coagulation cascade is a complex process that leads to the formation of a blood clot. There are two pathways that can lead to fibrin formation: the intrinsic pathway and the extrinsic pathway. The intrinsic pathway involves components that are already present in the blood and has a minor role in clotting. It is initiated by subendothelial damage, such as collagen, which leads to the formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and Factor 12. This complex activates Factor 11, which in turn activates Factor 9. Factor 9, along with its co-factor Factor 8a, forms the tenase complex, which activates Factor 10.

      The extrinsic pathway, on the other hand, requires tissue factor released by damaged tissue. This pathway is initiated by tissue damage, which leads to the binding of Factor 7 to tissue factor. This complex activates Factor 9, which works with Factor 8 to activate Factor 10. Both pathways converge at the common pathway, where activated Factor 10 causes the conversion of prothrombin to thrombin. Thrombin hydrolyses fibrinogen peptide bonds to form fibrin and also activates factor 8 to form links between fibrin molecules.

      Finally, fibrinolysis occurs, which is the process of clot resorption. Plasminogen is converted to plasmin to facilitate this process. It is important to note that certain factors are involved in both pathways, such as Factor 10, and that some factors are vitamin K dependent, such as Factors 2, 7, 9, and 10. The intrinsic pathway can be assessed by measuring the activated partial thromboplastin time (APTT), while the extrinsic pathway can be assessed by measuring the prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 54 - A pediatrician orders packed red cells for transfusion for a young patient with...

    Incorrect

    • A pediatrician orders packed red cells for transfusion for a young patient with significant blood loss. As you approach the storage unit, you notice that the power is off, which was keeping the red cells at the correct storage temperature.

      What temperature should this donation be stored at before use?

      Your Answer:

      Correct Answer: 4ºC

      Explanation:

      Guidelines for Red Blood Cell Transfusion

      In 2015, NICE released guidelines for the use of blood products, specifically red blood cells. These guidelines recommend different transfusion thresholds for patients with and without acute coronary syndrome (ACS). For patients without ACS, the transfusion threshold is 70 g/L, while for those with ACS, it is 80 g/L. The target hemoglobin level after transfusion is 70-90 g/L for patients without ACS and 80-100 g/L for those with ACS. It is important to note that these thresholds should not be used for patients with ongoing major hemorrhage or those who require regular blood transfusions for chronic anemia.

      When administering red blood cells, it is crucial to store them at 4°C prior to infusion. In non-urgent scenarios, a unit of RBC is typically transfused over a period of 90-120 minutes. By following these guidelines, healthcare professionals can ensure safe and effective transfusions for their patients.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 55 - A 65-year-old man presents to the clinic with a complaint of losing 1...

    Incorrect

    • A 65-year-old man presents to the clinic with a complaint of losing 1 stone in weight over the past three months. Apart from this, he has no significant medical history. During the physical examination, his abdomen is soft, and no palpable masses are detected. A normal PR examination is also observed. The patient's blood tests reveal a haemoglobin level of 80 g/L (120-160) and an MCV of 70 fL (80-96). What is the most appropriate initial investigation for this patient?

      Your Answer:

      Correct Answer: Upper GI endoscopy and colonoscopy

      Explanation:

      Possible GI Malignancy in a Man with Weight Loss and Microcytic Anaemia

      This man is experiencing weight loss and has an unexplained microcytic anaemia. The most probable cause of his blood loss is from the gastrointestinal (GI) tract, as there is no other apparent explanation. This could be due to an occult GI malignancy, which is why the recommended initial investigations are upper and lower GI endoscopy. These tests will help to identify any potential sources of bleeding in the GI tract and determine if there is an underlying malignancy. It is important to diagnose and treat any potential malignancy as early as possible to improve the patient’s prognosis. Therefore, prompt investigation and management are crucial in this case.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 56 - A six-month-old infant is brought to the emergency department due to sudden abdominal...

    Incorrect

    • A six-month-old infant is brought to the emergency department due to sudden abdominal pain and distension. The infant has a history of lethargy, growth restriction, and overall weakness. Upon abdominal examination, splenomegaly is noted. Further investigations reveal a diagnosis of sickle cell disease, with the acute presentation believed to be an acute crisis. Based on this information, what is the most probable haemoglobin trait in this patient?

      Your Answer:

      Correct Answer: HbS HbS

      Explanation:

      Understanding Sickle-Cell Anaemia

      Sickle-cell anaemia is a genetic disorder that occurs when an abnormal haemoglobin chain, known as HbS, is synthesized due to an autosomal recessive condition. This condition is more common in people of African descent, as the heterozygous condition offers some protection against malaria. In the UK, around 10% of Afro-Caribbean individuals are carriers of HbS. Symptoms in homozygotes typically do not develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.

      The pathophysiology of sickle-cell anaemia involves the substitution of the polar amino acid glutamate with the non-polar valine in each of the two beta chains (codon 6) of haemoglobin. This substitution decreases the water solubility of deoxy-Hb, causing HbS molecules to polymerize and sickle in the deoxygenated state. HbAS patients sickle at p02 2.5 – 4 kPa, while HbSS patients sickle at p02 5 – 6 kPa. Sickle cells are fragile and can cause haemolysis, block small blood vessels, and lead to infarction.

      To diagnose sickle-cell anaemia, haemoglobin electrophoresis is the definitive test. It is essential to understand the pathophysiology and symptoms of sickle-cell anaemia to provide appropriate care and management for affected individuals.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 57 - A 7-year-old boy is diagnosed by his pediatrician with a condition characterized by...

    Incorrect

    • A 7-year-old boy is diagnosed by his pediatrician with a condition characterized by a slightly low mean corpuscular volume (MCV) and a haemoglobin at the lower end of normal. Upon full investigation, it is discovered that he is missing a gene for one of his four alpha globin alleles. The doctor explains the condition to the boy and his parents, writing (aa/a-) to describe it. What is the name of this condition?

      Your Answer:

      Correct Answer: Silent carrier (alpha(+) heterozygous)

      Explanation:

      There are five potential disease phenotypes of alpha thalassaemia based on the number of faulty or missing globin alleles in a patient’s genotype. These include silent carrier (alpha(+) heterozygous) for one missing allele, alpha thalassaemia trait: alpha(0) heterozygous for two missing alleles, alpha thalassaemia trait: alpha(+) homozygous for two missing alleles, haemoglobin H disease for three missing alleles, and (–/–) for four missing alleles.

      Understanding Alpha-Thalassaemia

      Alpha-thalassaemia is a genetic disorder that results from a deficiency of alpha chains in haemoglobin. The condition is caused by a mutation in the alpha-globulin genes located on chromosome 16. The severity of the disease depends on the number of alpha globulin alleles affected. If one or two alleles are affected, the blood picture would be hypochromic and microcytic, but the haemoglobin level would typically be normal. However, if three alleles are affected, it results in a hypochromic microcytic anaemia with splenomegaly, which is known as Hb H disease. In the case of all four alleles being affected, which is known as homozygote, it can lead to death in utero, also known as hydrops fetalis or Bart’s hydrops. Understanding the different levels of severity of alpha-thalassaemia is crucial in diagnosing and managing the condition.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 58 - A 54-year-old man comes to the clinic complaining of fever and night sweats...

    Incorrect

    • A 54-year-old man comes to the clinic complaining of fever and night sweats that have been ongoing for several months. He reports a weight loss of 8 kg during this time and smokes half a pack of cigarettes per day. His temperature is 38 ºC, and he has splenomegaly on physical examination. No lymphadenopathy is observed. Laboratory results show a leukocyte count of 60 * 109, and a low leukocyte alkaline phosphatase level.

      What is the most likely finding in this patient?

      Your Answer:

      Correct Answer: t(9;22) translocation

      Explanation:

      Genetics of Haematological Malignancies

      Haematological malignancies are cancers that affect the blood, bone marrow, and lymphatic system. These cancers are often associated with specific genetic abnormalities, such as translocations. Here are some common translocations and their associated haematological malignancies:

      – Philadelphia chromosome (t(9;22)): This translocation is present in more than 95% of patients with chronic myeloid leukaemia (CML). It results in the fusion of the Abelson proto-oncogene with the BCR gene on chromosome 22, creating the BCR-ABL gene. This gene codes for a fusion protein with excessive tyrosine kinase activity, which is a poor prognostic indicator in acute lymphoblastic leukaemia (ALL).

      – t(15;17): This translocation is seen in acute promyelocytic leukaemia (M3) and involves the fusion of the PML and RAR-alpha genes.

      – t(8;14): Burkitt’s lymphoma is associated with this translocation, which involves the translocation of the MYC oncogene to an immunoglobulin gene.

      – t(11;14): Mantle cell lymphoma is associated with the deregulation of the cyclin D1 (BCL-1) gene.

      – t(14;18): Follicular lymphoma is associated with increased BCL-2 transcription due to this translocation.

      Understanding the genetic abnormalities associated with haematological malignancies is important for diagnosis, prognosis, and treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 59 - This full blood count (FBC) was obtained on a 60-year-old female who presents...

    Incorrect

    • This full blood count (FBC) was obtained on a 60-year-old female who presents with episodes of confusion, reports of visual hallucination and her neighbours say that she is withdrawn.

      Hb 139 g/L
      RBC 4.3 ×1012/L
      Hct 0.415
      MCV 98.5 fL
      MCH 32.8 pg
      Platelets 225 ×109/L
      WBC 8.01 ×109/L
      Neutrophils 4.67 ×109/L
      Lymphocytes 2.63 ×109/L
      Monocytes 0.22 ×109/L
      Eosinophils 0.05 ×109/L
      Basophils 0.04 ×109/L
      Others 0.10 ×109/L

      What is the most likely diagnosis based on the clinical history and full blood count results?

      Your Answer:

      Correct Answer: Alcohol withdrawal

      Explanation:

      Abnormalities on FBC and Possible Causes

      The FBC shows a normal Hb but an elevated MCV, which could be indicative of alcohol abuse. This is further supported by the patient’s increased confusion and withdrawal, suggesting acute withdrawal. Alcohol is known to cause an increase in MCV, while other causes such as B12 and folate deficiencies would also result in anemia. However, hypothyroidism and hematological malignancies are also associated with high MCV, but they are not likely causes in this clinical picture. Overall, the FBC abnormalities and clinical presentation suggest alcohol abuse and acute withdrawal as the most probable cause.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 60 - A toddler has been admitted following a prolonged chest infection. Recurrent chest and...

    Incorrect

    • A toddler has been admitted following a prolonged chest infection. Recurrent chest and gastrointestinal infections have plagued the child in their first two years of life. Blood antibody titres have revealed reduced levels of IgA, IgG, and IgE, while IgM is three times higher than normal. The patient is currently awaiting gene sequencing results to confirm the suspected diagnosis.

      Which gene mutations are responsible for the probable diagnosis?

      Your Answer:

      Correct Answer: CD40

      Explanation:

      Hyper IgM syndrome is caused by mutations in the CD40 gene, which affects the ability of B cells to produce immunoglobulin A, G, and E. While the production of IgM is still possible, the process of switching to other antibodies is impaired due to a lack of activated T-cells. This results in increased susceptibility to infections during early childhood. Treatment options include regular immunoglobulin, antibiotics, and granulocyte-colony stimulating factor (GCS-F).

      Overview of Primary Immunodeficiency Disorders

      Primary immunodeficiency disorders are conditions that affect the immune system’s ability to fight off infections and diseases. These disorders can be classified based on which component of the immune system is affected. Neutrophil disorders, for example, are caused by a lack of NADPH oxidase, which reduces the ability of phagocytes to produce reactive oxygen species. This leads to recurrent pneumonias and abscesses, particularly due to catalase-positive bacteria and fungi. B-cell disorders, on the other hand, are caused by defects in B cell development, resulting in low antibody levels and recurrent infections. T-cell disorders are caused by defects in T cell development, leading to recurrent viral and fungal diseases. Finally, combined B- and T-cell disorders are caused by defects in both B and T cell development, resulting in recurrent infections and an increased risk of malignancy. Understanding the underlying defects and symptoms of these disorders is crucial for proper diagnosis and treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 61 - When administering blood transfusions, certain patients may necessitate irradiated blood components to avoid...

    Incorrect

    • When administering blood transfusions, certain patients may necessitate irradiated blood components to avoid the development of transfusion-associated graft versus host disease (TA-GvHD).

      What distinguishes this specific blood product from standard blood products in terms of preventing TA-GvHD?

      Your Answer:

      Correct Answer: They are depleted in T-lymphocytes

      Explanation:

      Irradiated blood products are utilized because they have been stripped of T-lymphocytes, which can trigger severe reactions and even death if recognized as foreign agents by the host. This special requirement is particularly necessary for patients who are vulnerable to TA-GvHD, such as those with immune deficiencies or Hodgkin’s lymphoma. On the other hand, CMV negative blood products are used to minimize the risk of CMV transmission in neonates or immunocompromised individuals. In some cases, washed blood products may be ordered for patients who experience recurrent severe allergic transfusion reactions or urticarial reactions that are not prevented by pre-transfusion antihistamine and corticosteroid administration. It is important to note that the depletion of B-lymphocytes is not a primary reason for using irradiated blood products, and there is no evidence that irradiation reduces the risk of TA-GvHD by depleting eosinophil count.

      CMV Negative and Irradiated Blood Products

      Blood products that are CMV negative and irradiated are used in specific situations to prevent certain complications. CMV is a virus that is transmitted through leucocytes, but as most blood products are now leucocyte depleted, CMV negative products are not often needed. However, in situations where CMV transmission is a concern, such as in granulocyte transfusions, intra-uterine transfusions, neonates up to 28 days post expected date of delivery, bone marrow/stem cell transplants, immunocompromised patients, and those with/previous Hodgkin lymphoma, CMV negative blood products are used.

      On the other hand, irradiated blood products are depleted of T-lymphocytes and are used to prevent transfusion-associated graft versus host disease (TA-GVHD) caused by engraftment of viable donor T lymphocytes. Irradiated blood products are used in situations such as granulocyte transfusions, intra-uterine transfusions, neonates up to 28 days post expected date of delivery, bone marrow/stem cell transplants, and in patients who have received chemotherapy or have congenital immunodeficiencies.

      In summary, CMV negative and irradiated blood products are used in specific situations to prevent complications related to CMV transmission and TA-GVHD. The use of these blood products is determined based on the patient’s medical history and condition.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 62 - A 35-year-old obese nulliparous woman has given birth to a live singleton and...

    Incorrect

    • A 35-year-old obese nulliparous woman has given birth to a live singleton and the placenta, but is experiencing excessive bleeding. The registrar estimates that she has lost around 600 ml of blood. Despite receiving both crystalloid and colloid fluids, she remains haemodynamically unstable. As crossmatched blood is not yet available and her blood group is unknown, what blood group should be given to prevent a transfusion mismatch?

      Your Answer:

      Correct Answer: O negative

      Explanation:

      What is the blood group that can be given to anyone regardless of their blood type?

      Blood Products and Cell Saver Devices

      Blood products are essential in various medical procedures, especially in cases where patients require transfusions due to anaemia or bleeding. Packed red cells, platelet-rich plasma, platelet concentrate, fresh frozen plasma, and cryoprecipitate are some of the commonly used whole blood fractions. Fresh frozen plasma is usually administered to patients with clotting deficiencies, while cryoprecipitate is a rich source of Factor VIII and fibrinogen. Cross-matching is necessary for all blood products, and cell saver devices are used to collect and re-infuse a patient’s own blood lost during surgery.

      Cell saver devices come in two types, those that wash the blood cells before re-infusion and those that do not. The former is more expensive and complicated to operate but reduces the risk of re-infusing contaminated blood. The latter avoids the use of donor blood and may be acceptable to Jehovah’s witnesses. However, it is contraindicated in malignant diseases due to the risk of facilitating disease dissemination.

      In some surgical patients, the use of warfarin can pose specific problems and may require the use of specialised blood products. Warfarin reversal can be achieved through the administration of vitamin K, fresh frozen plasma, or human prothrombin complex. Fresh frozen plasma is used less commonly now as a first-line warfarin reversal, and human prothrombin complex is preferred due to its rapid action. However, it should be given with vitamin K as factor 6 has a short half-life.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 63 - A 44-year-old man presents with a widespread maculopapular rash and fever after undergoing...

    Incorrect

    • A 44-year-old man presents with a widespread maculopapular rash and fever after undergoing haematopoietic cell transplantation for multiple myeloma. The diagnosis is GVHD. What cell type is primarily responsible for the patient's symptoms?

      Your Answer:

      Correct Answer: Donor T cells

      Explanation:

      GVHD is a condition where T cells from the donor tissue (the graft) attack healthy cells in the recipient (the host). This can occur after a haematopoietic cell transplantation and is diagnosed based on symptoms such as fever, rash, and gastrointestinal issues. Antigen-presenting cells activate the donor T cells, but do not attack host cells. B cells, host T cells, and mast cells do not contribute to the attack on host tissue in GVHD.

      Understanding Graft Versus Host Disease

      Graft versus host disease (GVHD) is a complication that can occur after bone marrow or solid organ transplantation. It happens when the T cells in the donor tissue attack the recipient’s cells. This is different from transplant rejection, where the recipient’s immune cells attack the donor tissue. GVHD is diagnosed using the Billingham criteria, which require that the transplanted tissue contains functioning immune cells, the donor and recipient are immunologically different, and the recipient is immunocompromised.

      The incidence of GVHD varies, but it can occur in up to 50% of patients who receive allogeneic bone marrow transplants. Risk factors include poorly matched donor and recipient, the type of conditioning used before transplantation, gender disparity between donor and recipient, and the source of the graft.

      Acute and chronic GVHD are considered separate syndromes. Acute GVHD typically occurs within 100 days of transplantation and affects the skin, liver, and gastrointestinal tract. Chronic GVHD may occur after acute disease or arise de novo and has a more varied clinical picture.

      Diagnosis of GVHD is largely clinical and based on the exclusion of other pathology. Signs and symptoms of acute GVHD include a painful rash, jaundice, diarrhea, nausea, vomiting, and fever. Chronic GVHD can affect the skin, eyes, gastrointestinal tract, and lungs.

      Treatment of GVHD involves immunosuppression and supportive measures. Intravenous steroids are the mainstay of treatment for severe cases of acute GVHD, while extended courses of steroid therapy are often needed in chronic GVHD. Second-line therapies include anti-TNF, mTOR inhibitors, and extracorporeal photopheresis. Topical steroid therapy may be sufficient in mild disease with limited cutaneous involvement. However, excessive immunosuppression may increase the risk of infection and limit the beneficial graft-versus-tumor effect of the transplant.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 64 - A 45-year-old individual undergoes a lump excision procedure and upon examination of a...

    Incorrect

    • A 45-year-old individual undergoes a lump excision procedure and upon examination of a histological section, a clump of Reed Sternberg cells is identified by the pathologist. What is the probable diagnosis?

      Your Answer:

      Correct Answer: Hodgkins lymphoma

      Explanation:

      Understanding Hodgkin’s Lymphoma: Symptoms and Risk Factors

      Hodgkin’s lymphoma is a type of cancer that affects the lymphocytes and is characterized by the presence of Reed-Sternberg cells. It is most commonly seen in people in their third and seventh decades of life. There are certain risk factors that increase the likelihood of developing Hodgkin’s lymphoma, such as HIV and the Epstein-Barr virus.

      The most common symptom of Hodgkin’s lymphoma is lymphadenopathy, which is the enlargement of lymph nodes. This is usually painless, non-tender, and asymmetrical, and is most commonly seen in the neck, followed by the axillary and inguinal regions. In some cases, alcohol-induced lymph node pain may be present, but this is seen in less than 10% of patients. Other symptoms of Hodgkin’s lymphoma include weight loss, pruritus, night sweats, and fever (Pel-Ebstein). A mediastinal mass may also be present, which can cause symptoms such as coughing. In some cases, Hodgkin’s lymphoma may be found incidentally on a chest x-ray.

      When investigating Hodgkin’s lymphoma, normocytic anaemia may be present, which can be caused by factors such as hypersplenism, bone marrow replacement by HL, or Coombs-positive haemolytic anaemia. Eosinophilia may also be present, which is caused by the production of cytokines such as IL-5. LDH levels may also be raised.

      In summary, Hodgkin’s lymphoma is a type of cancer that affects the lymphocytes and is characterized by the presence of Reed-Sternberg cells. It is most commonly seen in people in their third and seventh decades of life and is associated with risk factors such as HIV and the Epstein-Barr virus. Symptoms of Hodgkin’s lymphoma include lymphadenopathy, weight loss, pruritus, night sweats, and fever. When investigating Hodgkin’s lymphoma, normocytic anaemia, eosinophilia, and raised LDH levels may be present.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 65 - A 62-year-old man presents to his GP with a complaint of lower back...

    Incorrect

    • A 62-year-old man presents to his GP with a complaint of lower back pain that has been bothering him for the past month. He denies any recent injury or trauma to his back. The pain is constant and is localized around the T12 and L1 vertebrae. Additionally, he has been experiencing night sweats and has lost around one stone in weight over the past two months, despite having a normal appetite. He also reports experiencing paraesthesia in the first three and a half digits of his right hand. What is the most probable cause of this patient's back pain?

      Your Answer:

      Correct Answer: Multiple myeloma

      Explanation:

      Multiple Myeloma and Carpal Tunnel Syndrome

      Multiple myeloma (MM) is a condition that results in the increased production of amyloid light chains, which can deposit in various organs, including the narrow carpal tunnel. This deposition can cause carpal tunnel syndrome, which is characterized by median nerve neuropathy. MM is caused by the clonal proliferation of monoclonal antibodies, which can lead to increased plasma volume and free light chains in the blood. These free light chains can then be processed into insoluble fibrillation proteins and deposited in various tissues throughout the body, resulting in amyloid deposits.

      It is important to note the ALARM signs and symptoms in the clinical history, such as unexplained weight loss and night sweats, which can indicate malignancy. In this case, MM and prostatic carcinoma are the two most likely options. However, the absence of urinary symptoms in this patient makes MM more likely. It is important to consider that an elderly gentleman presenting with low back pain could suggest secondary metastases to axial vertebral bone from primary prostatic carcinoma and should be high up on the list of differentials.

      In summary, carpal tunnel syndrome can be a result of amyloid deposition in the carpal tunnel due to MM. It is important to consider the ALARM signs and symptoms in the clinical history to determine the likelihood of malignancy, and to consider other potential causes of symptoms such as vertebral compression fracture.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 66 - A 25-year-old female comes to the clinic concerned about her risk of developing...

    Incorrect

    • A 25-year-old female comes to the clinic concerned about her risk of developing cancer due to her family history. Her grandfather recently passed away from lung cancer, and there are other cases of prostate, breast, and malignant melanoma in her family. She asks which type of cancer has the highest mortality rate in the UK. What is the correct answer?

      Your Answer:

      Correct Answer: Lung cancer

      Explanation:

      The leading cause of cancer deaths in the UK is lung cancer, while malignant melanoma does not rank in the top 10. Prostate cancer is the most prevalent cancer in men and the second most common cause of cancer-related deaths in men. Breast cancer is the second most common cause of cancer deaths in women.

      Cancer in the UK: Common Types and Causes of Death

      Cancer is a major health concern in the UK, with several types of cancer affecting a significant number of people. The most common types of cancer in the UK are breast, lung, colorectal, prostate, bladder, non-Hodgkin’s lymphoma, melanoma, stomach, oesophagus, and pancreas. However, when it comes to causes of death from cancer, lung cancer tops the list, followed by colorectal, breast, prostate, and pancreatic cancer. Other types of cancer that contribute to cancer-related deaths in the UK include oesophageal, stomach, bladder, non-Hodgkin’s lymphoma, and ovarian cancer. It is important to note that non-melanoma skin cancer is not included in these statistics. Despite the prevalence of cancer in the UK, there are various treatments and support available for those affected by the disease.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 67 - A 32-year-old female patient arrives at the emergency department with suspected pulmonary embolism....

    Incorrect

    • A 32-year-old female patient arrives at the emergency department with suspected pulmonary embolism. The patient has a history of multiple deep vein thromboses on separate occasions and has few risk factors for thrombosis, but a significant family history of DVT. The consultant suspects an inherited thrombophilia.

      What could be the possible diagnosis in this scenario?

      Your Answer:

      Correct Answer: Factor V Leiden

      Explanation:

      The most probable diagnosis for this case is factor V Leiden, which is the most common inherited thrombophilia. This condition causes resistance to activated protein C, which normally breaks down clotting factor V to prevent excessive clotting. As a result, individuals with factor V Leiden have an increased risk of developing blood clots, particularly deep vein thrombosis.

      Antiphospholipid syndrome is another thrombophilia, but it is an acquired autoimmune disorder that is less common than factor V Leiden. It is characterized by inappropriate clotting and miscarriage, which are not present in this case.

      Haemophilia A and von Willebrand disease are bleeding disorders that increase the risk of excessive bleeding, not clotting. Therefore, they are unlikely to be the cause of the patient’s thrombosis.

      Protein C deficiency has a similar mechanism and presentation to factor V Leiden, but it is less common. Hence, it is not the most probable diagnosis in this case.

      Thrombophilia is a condition that causes an increased risk of blood clots. It can be inherited or acquired. Inherited thrombophilia is caused by genetic mutations that affect the body’s natural ability to prevent blood clots. The most common cause of inherited thrombophilia is a gain of function polymorphism called factor V Leiden, which affects the protein that helps regulate blood clotting. Other genetic mutations that can cause thrombophilia include deficiencies of naturally occurring anticoagulants such as antithrombin III, protein C, and protein S. The prevalence and relative risk of venous thromboembolism (VTE) vary depending on the specific genetic mutation.

      Acquired thrombophilia can be caused by conditions such as antiphospholipid syndrome or the use of certain medications, such as the combined oral contraceptive pill. These conditions can affect the body’s natural ability to prevent blood clots and increase the risk of VTE. It is important to identify and manage thrombophilia to prevent serious complications such as deep vein thrombosis and pulmonary embolism.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 68 - Which of the following tumors is most likely to cause early para-aortic nodal...

    Incorrect

    • Which of the following tumors is most likely to cause early para-aortic nodal metastasis in younger patients?

      Your Answer:

      Correct Answer: Ovarian

      Explanation:

      The ovarian vessels directly branch from the aorta to supply ovarian tumours. Meanwhile, the internal and external iliac nodes are responsible for draining the cervix.

      Para-aortic Lymphadenopathy and its Association with Metastasis

      Para-aortic lymphadenopathy is a condition where the lymph nodes located near the aorta become enlarged due to the spread of cancer cells. This condition is commonly associated with the metastasis of cancer cells from various organs, including the testis, ovary, and uterine fundus. In these cases, the cancer cells spread to the para-aortic lymph nodes at an early stage of the disease.

      However, it is important to note that para-aortic nodal disease may also occur as a result of cancer cells spreading from other organs. In these cases, the para-aortic nodal disease represents a much later stage of the disease, as other nodal stations are involved earlier.

      Overall, para-aortic lymphadenopathy is a significant concern for individuals with cancer, as it can indicate the spread of cancer cells to other parts of the body. Early detection and treatment of para-aortic nodal disease can improve the chances of successful treatment and recovery.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 69 - A 67-year-old woman arrives at the emergency department complaining of sudden left leg...

    Incorrect

    • A 67-year-old woman arrives at the emergency department complaining of sudden left leg pain, redness, and swelling. She recently traveled from Australia and denies any history of trauma or family history of similar symptoms. What underlying risk factor may make her more susceptible to this condition?

      Your Answer:

      Correct Answer: Polycythaemia rubra vera

      Explanation:

      The risk of venous thromboembolism is elevated in individuals with polycythaemia due to the abnormal overproduction of red blood cells, which leads to increased blood viscosity and slower flow rate, increasing the likelihood of clot formation. Conversely, low BMI does not increase the risk of VTE, while obesity is a known risk factor. Additionally, thrombophilia, not haemophilia, is a risk factor for VTE.

      Risk Factors for Venous Thromboembolism

      Venous thromboembolism (VTE) is a condition where blood clots form in the veins, which can lead to serious complications such as pulmonary embolism (PE). While some common predisposing factors include malignancy, pregnancy, and the period following an operation, there are many other factors that can increase the risk of VTE. These include underlying conditions such as heart failure, thrombophilia, and nephrotic syndrome, as well as medication use such as the combined oral contraceptive pill and antipsychotics. It is important to note that around 40% of patients diagnosed with a PE have no major risk factors. Therefore, it is crucial to be aware of all potential risk factors and take appropriate measures to prevent VTE.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 70 - A 75-year-old man has recently been diagnosed with chronic myeloid leukaemia and is...

    Incorrect

    • A 75-year-old man has recently been diagnosed with chronic myeloid leukaemia and is undergoing chemotherapy. Following the start of treatment, he experiences acute renal failure and a significant increase in uric acid levels. What other electrolyte abnormality is likely to be present?

      Your Answer:

      Correct Answer: Hyperphosphataemia

      Explanation:

      The individual has been diagnosed with tumour lysis syndrome, which is a dangerous complication that can arise when commencing chemotherapy for cancer, particularly for lymphoma and leukaemia. Tumour lysis syndrome encompasses a range of metabolic imbalances, such as elevated levels of potassium, phosphates, and uric acid, as well as reduced levels of calcium. These imbalances can result in severe complications, including acute kidney failure, irregular heartbeats, and seizures.

      Understanding Tumour Lysis Syndrome

      Tumour lysis syndrome (TLS) is a life-threatening condition that can occur during the treatment of high-grade lymphomas and leukaemias. It is caused by the breakdown of tumour cells and the release of chemicals into the bloodstream. While it can occur without chemotherapy, it is usually triggered by the introduction of combination chemotherapy. Patients at high risk of TLS should be given prophylactic medication such as IV allopurinol or IV rasburicase to prevent the potentially deadly effects of tumour cell lysis.

      TLS leads to a high potassium and high phosphate level in the presence of a low calcium. It should be suspected in any patient presenting with an acute kidney injury in the presence of a high phosphate and high uric acid level. From 2004, TLS has been graded using the Cairo-Bishop scoring system, which takes into account laboratory and clinical factors.

      It is important to be aware of TLS and take preventative measures to avoid its potentially fatal consequences. By understanding the causes and symptoms of TLS, healthcare professionals can provide appropriate treatment and improve patient outcomes.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 71 - In the majority of cases of transfusion reaction caused by Rh antibodies, what...

    Incorrect

    • In the majority of cases of transfusion reaction caused by Rh antibodies, what is the probable Rh grouping of the mother, father, and child?

      Your Answer:

      Correct Answer: The mother is Rh-negative, the father Rh-positive, and the baby Rh-positive

      Explanation:

      Transfusion Reactions and the Role of Rh Factor

      A transfusion reaction can occur when Rh-positive blood is given to a person who is Rh-negative and has been previously exposed to Rh-positive blood. This exposure can result in the development of anti-Rh antibodies, which can cause a reaction when Rh-positive blood is introduced into the body. In addition to transfusions, the Rh factor can also play a role in pregnancy. If a mother is Rh-negative and the father and baby are Rh-positive, there is a risk of a transfusion reaction occurring in the fetus or newborn, leading to a condition known as hemolytic disease of the fetus and newborn (HDFN). It is important to take preventative measures to avoid transfusion reactions and HDFN, such as ensuring blood compatibility and administering Rh immune globulin to Rh-negative mothers during pregnancy. the role of the Rh factor can help prevent these potentially dangerous reactions.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 72 - A 30-year-old patient presents with a pale complexion and a tendency to bruise...

    Incorrect

    • A 30-year-old patient presents with a pale complexion and a tendency to bruise easily. Upon consultation with their GP, a blood test is ordered.

      WBC count: 6.0 x 109/L
      Neutrophil count: 0.9 x 109/L

      Which type of leukemia is typically linked to these blood test results?

      Your Answer:

      Correct Answer: Acute myeloid leukaemia

      Explanation:

      The patient is exhibiting symptoms of anaemia and low platelets, as evidenced by their pallor and bruising. Their blood tests indicate low levels of neutrophils, but normal levels of white cells. This suggests that there may be an issue with the patient’s common myeloid progenitor cells, as neutrophils, erythrocytes, and platelets all originate from this lineage. Therefore, options 1, 3, and 5 are incorrect, as they involve cancers that affect the lymphoid lineage. Acute myeloid leukaemia can cause low levels of myeloid cells due to a differentiation block, while chronic myeloid leukaemia can cause elevated neutrophil levels as it does not exhibit a differentiation block.

      Haematopoiesis: The Generation of Immune Cells

      Haematopoiesis is the process by which immune cells are produced from haematopoietic stem cells in the bone marrow. These stem cells give rise to two main types of progenitor cells: myeloid and lymphoid progenitor cells. All immune cells are derived from these progenitor cells.

      The myeloid progenitor cells generate cells such as macrophages/monocytes, dendritic cells, neutrophils, eosinophils, basophils, and mast cells. On the other hand, lymphoid progenitor cells give rise to T cells, NK cells, B cells, and dendritic cells.

      This process is essential for the proper functioning of the immune system. Without haematopoiesis, the body would not be able to produce the necessary immune cells to fight off infections and diseases. Understanding haematopoiesis is crucial in developing treatments for diseases that affect the immune system.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 73 - A 55-year-old male presents with fever, night sweats, weight loss, and upper abdominal...

    Incorrect

    • A 55-year-old male presents with fever, night sweats, weight loss, and upper abdominal pain. Upon examination, his liver and spleen are enlarged. A complete blood count shows elevated levels of basophils, eosinophils, and neutrophils. What is the most probable cause of these symptoms?

      Your Answer:

      Correct Answer: BCR-ABL

      Explanation:

      The symptoms displayed by this individual suggest the presence of chronic myeloid leukemia (CML), which is identified by the Philadelphia chromosome. This chromosome results from a genetic abnormality where chromosome 9 and 22 exchange genetic material, leading to the formation of the BCR-ABL gene.

      Understanding Chronic Myeloid Leukaemia and its Management

      Chronic myeloid leukaemia (CML) is a type of cancer that affects the blood and bone marrow. It is characterized by the presence of the Philadelphia chromosome in more than 95% of patients. This chromosome is formed due to a translocation between chromosomes 9 and 22, resulting in the fusion of the ABL proto-oncogene and the BCR gene. The resulting BCR-ABL gene produces a fusion protein that has excessive tyrosine kinase activity.

      CML typically affects individuals between the ages of 60-70 years and presents with symptoms such as anaemia, weight loss, sweating, and splenomegaly. The condition is also associated with an increase in granulocytes at different stages of maturation and thrombocytosis. In some cases, CML may undergo blast transformation, leading to acute myeloid leukaemia (AML) or acute lymphoblastic leukaemia (ALL).

      The management of CML involves various treatment options, including imatinib, which is considered the first-line treatment. Imatinib is an inhibitor of the tyrosine kinase associated with the BCR-ABL defect and has a very high response rate in chronic phase CML. Other treatment options include hydroxyurea, interferon-alpha, and allogenic bone marrow transplant. With proper management, individuals with CML can lead a normal life.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 74 - Which one of the following statements about the spleen is false? ...

    Incorrect

    • Which one of the following statements about the spleen is false?

      Your Answer:

      Correct Answer: The spleen is derived from endodermal tissue.

      Explanation:

      The spleen, which weighs 7oz (150-200g), is approximately 1 inch thick, 3 inches wide, and 5 inches long. It is located between the 9th and 11th ribs. While most of the gut is derived from endodermal tissue, the spleen is unique in that it originates from mesenchymal tissue.

      The Anatomy and Function of the Spleen

      The spleen is an organ located in the left upper quadrant of the abdomen. Its size can vary depending on the amount of blood it contains, but the typical adult spleen is 12.5cm long and 7.5cm wide, with a weight of 150g. The spleen is almost entirely covered by peritoneum and is separated from the 9th, 10th, and 11th ribs by both diaphragm and pleural cavity. Its shape is influenced by the state of the colon and stomach, with gastric distension causing it to resemble an orange segment and colonic distension causing it to become more tetrahedral.

      The spleen has two folds of peritoneum that connect it to the posterior abdominal wall and stomach: the lienorenal ligament and gastrosplenic ligament. The lienorenal ligament contains the splenic vessels, while the short gastric and left gastroepiploic branches of the splenic artery pass through the layers of the gastrosplenic ligament. The spleen is in contact with the phrenicocolic ligament laterally.

      The spleen has two main functions: filtration and immunity. It filters abnormal blood cells and foreign bodies such as bacteria, and produces properdin and tuftsin, which help target fungi and bacteria for phagocytosis. The spleen also stores 40% of platelets, reutilizes iron, and stores monocytes. Disorders of the spleen include massive splenomegaly, myelofibrosis, chronic myeloid leukemia, visceral leishmaniasis, malaria, Gaucher’s syndrome, portal hypertension, lymphoproliferative disease, haemolytic anaemia, infection, infective endocarditis, sickle-cell, thalassaemia, and rheumatoid arthritis.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 75 - A 26-year-old male is found to have Hodgkin's lymphoma. Which subtype is linked...

    Incorrect

    • A 26-year-old male is found to have Hodgkin's lymphoma. Which subtype is linked with the most positive outcome?

      Your Answer:

      Correct Answer: Classical lymphocyte predominant

      Explanation:

      The prognosis for the classical lymphocyte predominant variant is the most favorable, while the nodular lymphocyte predominant disease has a different disease entity and does not share the same positive prognosis.

      Understanding Hodgkin’s Lymphoma: Staging and Treatment

      Hodgkin’s lymphoma is a type of cancer that affects the lymphatic system. It is characterized by the presence of Reed-Sternberg cells, which are malignant lymphocytes. This type of cancer is most commonly seen in people in their third and seventh decades of life.

      To determine the extent of the cancer, doctors use the Ann-Arbor staging system. This system divides the cancer into four stages, with each stage being further divided into A or B. Stage I involves a single lymph node, while stage II involves two or more lymph nodes on the same side of the diaphragm. Stage III involves nodes on both sides of the diaphragm, and stage IV involves the spread of cancer beyond the lymph nodes.

      The main treatment for Hodgkin’s lymphoma is chemotherapy. Two combinations of drugs may be used: ABVD and BEACOPP. ABVD is considered the standard regime, while BEACOPP has better remission rates but higher toxicity. Radiotherapy and combined modality therapy (CMT) may also be used. In some cases, hematopoietic cell transplantation may be used for relapsed or refractory classic Hodgkin lymphoma.

      While most patients now achieve long-term survival free of Hodgkin’s lymphoma with modern therapy, complications of treatment are a concern. Secondary malignancies, particularly solid tumors such as breast and lung cancer, are a risk for these patients. It is important for patients to discuss the potential risks and benefits of treatment with their healthcare team.

      Overall, understanding the staging and treatment options for Hodgkin’s lymphoma can help patients and their families make informed decisions about their care.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 76 - A 67-year-old woman complains of feeling tired and dizzy. During the examination, she...

    Incorrect

    • A 67-year-old woman complains of feeling tired and dizzy. During the examination, she appears pale and has an enlarged spleen and liver. She has been consuming a bottle of wine daily for the past 25 years.

      Her blood work reveals:

      Hemoglobin (Hb) level of 72 g/L (normal range for females: 115 - 160)
      Mean Cell Volume (MCV) of 73 fL (normal range: 80 - 100)
      Ferritin level of 410 ng/mL (normal range: 10 - 300)
      Blood film shows basophilic stippling of red blood cells

      What is the most probable diagnosis?

      Your Answer:

      Correct Answer: Sideroblastic anaemia

      Explanation:

      The correct answer is sideroblastic anaemia, which is characterized by hypochromic microcytic anaemia, high levels of ferritin iron and transferrin saturation, and the presence of basophilic stippling in red blood cells. This condition occurs when haem formation is incomplete, leading to the accumulation of iron in the mitochondria and the formation of a ring sideroblast around the nucleus. Alcohol consumption is a common cause, and treatment is supportive.

      B12 deficiency is a type of megaloblastic anaemia, which results in a high mean corpuscular volume (MCV). It is typically caused by conditions that lead to vitamin B12 malabsorption, such as autoimmune gastritis.

      Iron deficiency is a type of microcytic anaemia, which is characterized by a low MCV. However, in iron deficiency, the ferritin level is typically low, and pencil-shaped cells may be present in the blood film.

      Sickle cell anaemia is a normochromic-normocytic haemolytic disorder, so the MCV should be normal. Patients often have a positive family history, and the blood film may show sickle cells and features of hyposplenism, such as target cells and Howell-Jolly bodies.

      Understanding Sideroblastic Anaemia

      Sideroblastic anaemia is a medical condition that occurs when red blood cells fail to produce enough haem, which is partly synthesized in the mitochondria. This results in the accumulation of iron in the mitochondria, forming a ring around the nucleus known as a ring sideroblast. The condition can be either congenital or acquired.

      The congenital cause of sideroblastic anaemia is delta-aminolevulinate synthase-2 deficiency. On the other hand, acquired causes include myelodysplasia, alcohol, lead, and anti-TB medications.

      To diagnose sideroblastic anaemia, doctors may conduct a full blood count, iron studies, and a blood film. The results may show hypochromic microcytic anaemia, high ferritin, high iron, high transferrin saturation, and basophilic stippling of red blood cells. A bone marrow test may also be done, and Prussian blue staining can reveal ringed sideroblasts.

      Management of sideroblastic anaemia is mainly supportive, and treatment focuses on addressing any underlying cause. Pyridoxine may also be prescribed to help manage the condition.

      In summary, sideroblastic anaemia is a condition that affects the production of haem in red blood cells, leading to the accumulation of iron in the mitochondria. It can be congenital or acquired, and diagnosis involves various tests. Treatment is mainly supportive, and addressing any underlying cause is crucial.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 77 - A 75-year-old man experiences an urgent amputation due to severe sepsis and gangrene...

    Incorrect

    • A 75-year-old man experiences an urgent amputation due to severe sepsis and gangrene in his lower limbs. Following the surgery, he develops disseminated intravascular coagulation. Which clotting factor will be depleted the fastest during this process?

      Your Answer:

      Correct Answer: Factor V and VIII

      Explanation:

      D-I-S-S-E-M-I-N-A-T-E-D

      R-Rewritten
      E-Explanations
      W-Widespread
      R-Reporting
      I-Information
      T-Transmission
      E-Exposure

      M-Multiple sources
      E-Extensive dissemination
      D-Distribution

      Rewriting and disseminating information can help to ensure that it is widely understood and accessible. This can be especially important in cases where there are multiple sources of information or when the information needs to be widely distributed. In some cases, such as with DIC, disseminating information can be critical for understanding and treating the condition.

      Disseminated Intravascular Coagulation: A Condition of Simultaneous Coagulation and Haemorrhage

      Disseminated intravascular coagulation (DIC) is a medical condition characterized by simultaneous coagulation and haemorrhage. It is caused by the initial formation of thrombi that consume clotting factors and platelets, ultimately leading to bleeding. DIC can be caused by various factors such as infection, malignancy, trauma, liver disease, and obstetric complications.

      Clinically, bleeding is usually the dominant feature of DIC, accompanied by bruising, ischaemia, and organ failure. Blood tests can reveal prolonged clotting times, thrombocytopenia, decreased fibrinogen, and increased fibrinogen degradation products. The treatment of DIC involves addressing the underlying cause and providing supportive management.

      In summary, DIC is a serious medical condition that requires prompt diagnosis and management. It is important to identify the underlying cause and provide appropriate treatment to prevent further complications. With proper care and management, patients with DIC can recover and regain their health.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 78 - A 65-year-old smoker visits his doctor complaining of a persistent mouth ulcer that...

    Incorrect

    • A 65-year-old smoker visits his doctor complaining of a persistent mouth ulcer that has not healed with conventional treatment for the past 3 months. The ulcer is situated in the middle of the right side of his tongue. Upon biopsy, it is revealed that the ulcer is a squamous cell carcinoma. Further examination is conducted to determine the possibility of lymphatic spread.

      What is the primary group of regional lymph nodes that may be affected by the spread of this tumor?

      Your Answer:

      Correct Answer: Submandibular

      Explanation:

      The submandibular lymph nodes are the primary drainage site for the mid-portion of the tongue. Subsequently, the lymphatic fluid will spread to the deep cervical lymph nodes.

      Lymphatic Drainage of the Tongue

      The lymphatic drainage of the tongue varies depending on the location of the tumour. The anterior two-thirds of the tongue have minimal communication of lymphatics across the midline, resulting in metastasis to the ipsilateral nodes being more common. On the other hand, the posterior third of the tongue has communicating networks, leading to early bilateral nodal metastases being more common in this area.

      The tip of the tongue drains to the submental nodes and then to the deep cervical nodes, while the mid portion of the tongue drains to the submandibular nodes and then to the deep cervical nodes. If mid tongue tumours are laterally located, they will usually drain to the ipsilateral deep cervical nodes. However, those from more central regions may have bilateral deep cervical nodal involvement. Understanding the lymphatic drainage of the tongue is crucial in determining the spread of tumours and planning appropriate treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 79 - A 10-year-old boy arrives at the emergency department with sudden onset of rapid...

    Incorrect

    • A 10-year-old boy arrives at the emergency department with sudden onset of rapid breathing. He has a history of cough and dehydration over the past 4 days. Upon respiratory examination, tachypnea is noted but no other significant findings. The child undergoes a series of tests and is ultimately diagnosed with a vaso-occlusive crisis due to mild sickle cell disease.

      What is the most probable haemoglobin trait in this patient?

      Your Answer:

      Correct Answer: HbA HbS

      Explanation:

      Understanding Sickle-Cell Anaemia

      Sickle-cell anaemia is a genetic disorder that occurs when an abnormal haemoglobin chain, known as HbS, is synthesized due to an autosomal recessive condition. This condition is more common in people of African descent, as the heterozygous condition offers some protection against malaria. In the UK, around 10% of Afro-Caribbean individuals are carriers of HbS. Symptoms in homozygotes typically do not develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.

      The pathophysiology of sickle-cell anaemia involves the substitution of the polar amino acid glutamate with the non-polar valine in each of the two beta chains (codon 6) of haemoglobin. This substitution decreases the water solubility of deoxy-Hb, causing HbS molecules to polymerize and sickle in the deoxygenated state. HbAS patients sickle at p02 2.5 – 4 kPa, while HbSS patients sickle at p02 5 – 6 kPa. Sickle cells are fragile and can cause haemolysis, block small blood vessels, and lead to infarction.

      To diagnose sickle-cell anaemia, haemoglobin electrophoresis is the definitive test. It is essential to understand the pathophysiology and symptoms of sickle-cell anaemia to provide appropriate care and management for affected individuals.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 80 - A 47-year-old male with alcoholic cirrhosis and decompensated liver failure is wondering which...

    Incorrect

    • A 47-year-old male with alcoholic cirrhosis and decompensated liver failure is wondering which clotting factor is least likely to be affected.

      Your Answer:

      Correct Answer: Factor VIII

      Explanation:

      Factor VIII is produced in the endothelial cells located in the liver, which makes it less susceptible to the impact of liver dysfunction.

      Abnormal coagulation can be caused by various factors such as heparin, warfarin, disseminated intravascular coagulation (DIC), and liver disease. Heparin prevents the activation of factors 2, 9, 10, and 11, while warfarin affects the synthesis of factors 2, 7, 9, and 10. DIC affects factors 1, 2, 5, 8, and 11, and liver disease affects factors 1, 2, 5, 7, 9, 10, and 11.

      When interpreting blood clotting test results, different disorders can be identified based on the levels of activated partial thromboplastin time (APTT), prothrombin time (PT), and bleeding time. Haemophilia is characterized by increased APTT levels, normal PT levels, and normal bleeding time. On the other hand, von Willebrand’s disease is characterized by increased APTT levels, normal PT levels, and increased bleeding time. Lastly, vitamin K deficiency is characterized by increased APTT and PT levels, and normal bleeding time. Proper interpretation of these results is crucial in diagnosing and treating coagulation disorders.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 81 - A 56-year-old man from Somalia is admitted to the nephrology ward due to...

    Incorrect

    • A 56-year-old man from Somalia is admitted to the nephrology ward due to acute-on-chronic kidney disease. He also has a known antithrombin III deficiency related to his chronic kidney disease. As part of his treatment, he is prescribed antithrombotic prophylaxis.

      What is the specific factor inhibited by antithrombin III?

      Your Answer:

      Correct Answer: Factors II, IX and X

      Explanation:

      Understanding Antithrombin III Deficiency

      Antithrombin III deficiency is a genetic condition that affects approximately 1 in 3,000 people. It is inherited in an autosomal dominant manner. This condition occurs when the body does not produce enough antithrombin III, a protein that helps to prevent blood clots by inhibiting certain clotting factors. Some patients with this deficiency have a shortage of normal antithrombin III, while others produce abnormal antithrombin III.

      People with antithrombin III deficiency are at an increased risk of developing recurrent venous thromboses, which are blood clots that form in the veins. While arterial thromboses can also occur, they are less common. To manage this condition, patients may need to take warfarin for the rest of their lives to prevent thromboembolic events. During pregnancy, heparin may be used instead. Antithrombin III concentrates may also be used during surgery or childbirth.

      It is important to note that patients with antithrombin III deficiency have a degree of resistance to heparin, so anti-Xa levels should be monitored carefully to ensure adequate anticoagulation. Compared to other inherited thrombophilias, antithrombin III deficiency is less common but has a higher relative risk of venous thromboembolism. Understanding this condition and its management is crucial for those affected and their healthcare providers.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 82 - A 50-year-old male patient arrives at the emergency department with abrupt chest pain...

    Incorrect

    • A 50-year-old male patient arrives at the emergency department with abrupt chest pain that worsens with deep breathing. The patient appears visibly agitated and short of breath, and coughs up a small quantity of blood while waiting. During the examination, the patient displays tachypnea and a red, swollen left leg. An ECG shows sinus tachycardia.

      What is the most significant risk factor for this patient's presentation?

      Your Answer:

      Correct Answer: Prostate malignancy

      Explanation:

      Having a malignancy increases the likelihood of developing pulmonary embolism, as all types of cancer are known to increase the risk of venous thromboembolism. However, bronchiectasis, despite causing breathlessness and haemoptysis, is less likely to result in an acute attack and is not a common risk factor for PE. Contrary to popular belief, individuals with a high BMI are more likely to develop blood clots than those with a low BMI. Finally, conditions 4 and 5 are not typically associated with an increased risk of pulmonary embolism.

      Risk Factors for Venous Thromboembolism

      Venous thromboembolism (VTE) is a condition where blood clots form in the veins, which can lead to serious complications such as pulmonary embolism (PE). While some common predisposing factors include malignancy, pregnancy, and the period following an operation, there are many other factors that can increase the risk of VTE. These include underlying conditions such as heart failure, thrombophilia, and nephrotic syndrome, as well as medication use such as the combined oral contraceptive pill and antipsychotics. It is important to note that around 40% of patients diagnosed with a PE have no major risk factors. Therefore, it is crucial to be aware of all potential risk factors and take appropriate measures to prevent VTE.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 83 - A 56-year-old man is sent home following an uneventful left total hip replacement....

    Incorrect

    • A 56-year-old man is sent home following an uneventful left total hip replacement. He has been prescribed a direct factor Xa inhibitor for thromboprophylaxis.

      What is the probable medication that the patient has been prescribed?

      Your Answer:

      Correct Answer: Apixaban

      Explanation:

      Apixaban directly inhibits factor Xa, while bivalirudin and dabigatran directly inhibit thrombin. On the other hand, enoxaparin is a type of low molecular weight heparin that indirectly inhibits factor Xa by forming a complex with antithrombin III, leading to irreversible inactivation of factor Xa.

      Direct oral anticoagulants (DOACs) are medications used to prevent stroke in non-valvular atrial fibrillation (AF), as well as for the prevention and treatment of venous thromboembolism (VTE). To be prescribed DOACs for stroke prevention, patients must have certain risk factors, such as a prior stroke or transient ischaemic attack, age 75 or older, hypertension, diabetes mellitus, or heart failure. There are four DOACs available, each with a different mechanism of action and method of excretion. Dabigatran is a direct thrombin inhibitor, while rivaroxaban, apixaban, and edoxaban are direct factor Xa inhibitors. The majority of DOACs are excreted either through the kidneys or the liver, with the exception of apixaban and edoxaban, which are excreted through the feces. Reversal agents are available for dabigatran and rivaroxaban, but not for apixaban or edoxaban.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 84 - A 45-year-old woman presents to her GP with a four-week history of dysphagia,...

    Incorrect

    • A 45-year-old woman presents to her GP with a four-week history of dysphagia, anorexia and weight loss. She has had a hoarse voice for several months.

      She has a 40 pack-year smoking history, starting from the age of 16 years. She drinks 30 units a week in the form of binge drinking beer over the weekend. She admits to having a very poor diet consisting mostly of fish and chips. She is noted to have a body mass index of 38kg/m².

      The GP refers her under a two-week wait for suspicion of oesophageal cancer.

      What risk factors contributed to her increased likelihood of developing this disease?

      Your Answer:

      Correct Answer: Nitrosamines

      Explanation:

      Exposure to nitrosamines is a known risk factor for the development of oesophageal and gastric cancer, particularly squamous cell carcinoma of the oesophagus. Nitrosamines are present in high levels in cigarette smoke, which is a significant source of exposure for this patient. Binge drinking of beer can also lead to high levels of nitrosamine exposure. Additionally, nitrosamines can be found in certain fried foods, such as fish and chips, as well as some cheeses.

      Aflatoxin, which is produced by Aspergillus species, is another known risk factor for cancer. Specifically, it increases the risk of developing hepatocellular carcinoma.

      Aniline dyes, which are commonly used in industrial dyeing and the rubber industry, have been linked to an increased risk of developing transitional cell carcinoma of the bladder.

      Asbestos, which was once widely used in insulation, building materials, and construction, is a well-known carcinogen that increases the risk of developing mesothelioma and bronchial cancers.

      Understanding Carcinogens and Their Link to Cancer

      Carcinogens are substances that have the potential to cause cancer. These substances can be found in various forms, including chemicals, radiation, and viruses. Aflatoxin, which is produced by Aspergillus, is a carcinogen that can cause liver cancer. Aniline dyes, on the other hand, can lead to bladder cancer, while asbestos is known to cause mesothelioma and bronchial carcinoma. Nitrosamines are another type of carcinogen that can cause oesophageal and gastric cancer, while vinyl chloride can lead to hepatic angiosarcoma.

      It is important to understand the link between carcinogens and cancer, as exposure to these substances can increase the risk of developing the disease. By identifying and avoiding potential carcinogens, individuals can take steps to reduce their risk of cancer. Additionally, researchers continue to study the effects of various substances on the body, in order to better understand the mechanisms behind cancer development and to develop new treatments and prevention strategies. With continued research and education, it is possible to reduce the impact of carcinogens on human health.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 85 - A 22-year-old male arrives at the emergency department with excessive epistaxis. Despite applying...

    Incorrect

    • A 22-year-old male arrives at the emergency department with excessive epistaxis. Despite applying pressure on the anterior nares for the past four hours, the bleeding has not stopped. Nasal packing has also failed to control the bleeding. The on-call ENT specialist administers topical tranexamic acid to a visibly bleeding artery, which results in a reduction in bleeding.

      What is the mode of action of tranexamic acid?

      Your Answer:

      Correct Answer: Prevents plasmin from breaking down fibrin clots

      Explanation:

      Tranexamic acid prevents major haemorrhage by binding to plasminogen and preventing plasmin from breaking down fibrin clots. Its mechanism of action is not related to increasing the availability of vitamin K or inhibiting anticlotting factors protein C and S. Similarly, reducing the availability of vitamin K would not be the mechanism of action of tranexamic acid. While stimulating anticlotting factors protein C and S would maintain clots, it is not the mechanism of action of tranexamic acid.

      Understanding Tranexamic Acid

      Tranexamic acid is a synthetic derivative of lysine that acts as an antifibrinolytic. Its primary function is to bind to lysine receptor sites on plasminogen or plasmin, preventing plasmin from degrading fibrin. This medication is commonly prescribed to treat menorrhagia.

      In addition to its use in treating menorrhagia, tranexamic acid has been investigated for its role in trauma. The CRASH 2 trial found that administering tranexamic acid within the first 3 hours of bleeding trauma can be beneficial. In cases of major haemorrhage, tranexamic acid is given as an IV bolus followed by an infusion.

      Ongoing research is also exploring the potential of tranexamic acid in treating traumatic brain injury. Overall, tranexamic acid is a medication with important applications in managing bleeding disorders and trauma.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 86 - A 33-year-old female patient complained of pain and bleeding during sexual intercourse. Upon...

    Incorrect

    • A 33-year-old female patient complained of pain and bleeding during sexual intercourse. Upon referral to colposcopy, she was diagnosed with cervical cancer. The doctor informed her that she would need to undergo surgery to remove the tumour and also remove the lymph nodes that drain the cervix.

      Which group of lymph nodes is the doctor referring to?

      Your Answer:

      Correct Answer: Internal iliac nodes

      Explanation:

      The cervix primarily drains into the internal iliac lymph nodes. The deep inguinal lymph nodes do not drain the cervix, but they do drain the clitoris and glans penis. The external iliac lymph nodes are not significantly involved in the lymphatic drainage of the cervix, but they do play a role in the drainage of the bladder fundus, prostate, and adductor region of the thigh. The para-aortic nodes drain the ovaries, but not the cervix. The superficial inguinal lymph nodes are not involved in the drainage of the cervix, but they are important in the drainage of the anal canal (below the pectinate line), scrotum, and perineum.

      Lymphatic drainage is the process by which lymphatic vessels carry lymph, a clear fluid containing white blood cells, away from tissues and organs and towards lymph nodes. The lymphatic vessels that drain the skin and follow venous drainage are called superficial lymphatic vessels, while those that drain internal organs and structures follow the arteries and are called deep lymphatic vessels. These vessels eventually lead to lymph nodes, which filter and remove harmful substances from the lymph before it is returned to the bloodstream.

      The lymphatic system is divided into two main ducts: the right lymphatic duct and the thoracic duct. The right lymphatic duct drains the right side of the head and right arm, while the thoracic duct drains everything else. Both ducts eventually drain into the venous system.

      Different areas of the body have specific primary lymph node drainage sites. For example, the superficial inguinal lymph nodes drain the anal canal below the pectinate line, perineum, skin of the thigh, penis, scrotum, and vagina. The deep inguinal lymph nodes drain the glans penis, while the para-aortic lymph nodes drain the testes, ovaries, kidney, and adrenal gland. The axillary lymph nodes drain the lateral breast and upper limb, while the internal iliac lymph nodes drain the anal canal above the pectinate line, lower part of the rectum, and pelvic structures including the cervix and inferior part of the uterus. The superior mesenteric lymph nodes drain the duodenum and jejunum, while the inferior mesenteric lymph nodes drain the descending colon, sigmoid colon, and upper part of the rectum. Finally, the coeliac lymph nodes drain the stomach.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 87 - A 60-year-old male visits his doctor complaining of a lump on the side...

    Incorrect

    • A 60-year-old male visits his doctor complaining of a lump on the side of his neck. He reports feeling exhausted and experiencing night sweats. Following a needle core biopsy, the patient is diagnosed with follicular lymphoma. Which chromosomes are linked to this condition through translocation?

      Your Answer:

      Correct Answer: 14 and 18

      Explanation:

      The translocation of chromosomes is associated with various types of lymphoma and leukaemia. For example, the t(14;18) translocation causes follicular lymphoma by increasing BCL-2 transcription. Similarly, the t(8;14) translocation causes Burkitt lymphoma, while the t(9;22) translocation leads to the Philadelphia chromosome and chronic myeloid leukaemia. Mantle cell lymphoma is associated with the t(11;14) translocation. These translocations can help diagnose and classify these haematological malignancies.

      Genetics of Haematological Malignancies

      Haematological malignancies are cancers that affect the blood, bone marrow, and lymphatic system. These cancers are often associated with specific genetic abnormalities, such as translocations. Here are some common translocations and their associated haematological malignancies:

      – Philadelphia chromosome (t(9;22)): This translocation is present in more than 95% of patients with chronic myeloid leukaemia (CML). It results in the fusion of the Abelson proto-oncogene with the BCR gene on chromosome 22, creating the BCR-ABL gene. This gene codes for a fusion protein with excessive tyrosine kinase activity, which is a poor prognostic indicator in acute lymphoblastic leukaemia (ALL).

      – t(15;17): This translocation is seen in acute promyelocytic leukaemia (M3) and involves the fusion of the PML and RAR-alpha genes.

      – t(8;14): Burkitt’s lymphoma is associated with this translocation, which involves the translocation of the MYC oncogene to an immunoglobulin gene.

      – t(11;14): Mantle cell lymphoma is associated with the deregulation of the cyclin D1 (BCL-1) gene.

      – t(14;18): Follicular lymphoma is associated with increased BCL-2 transcription due to this translocation.

      Understanding the genetic abnormalities associated with haematological malignancies is important for diagnosis, prognosis, and treatment.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 88 - A 50-year-old female is brought to the emergency department by an ambulance after...

    Incorrect

    • A 50-year-old female is brought to the emergency department by an ambulance after she was found collapsed on the street by a bystander. Within a few minutes of arrival she developed severe abdominal pain and became severely agitated.

      Her respiratory rate is 35 breaths per minute, heart rate 110 beats per minute, temperature 39.3ºC. Her prothrombin time and activated partial thromboplastin time are increased, and her fibrinogen levels are lower than normal. Her D-dimer is positive.

      Hb 96 g/l
      Platelets 85 * 109/l
      WBC 14 * 109/l

      Blood smears are sent to the laboratory.

      What is most likely to be seen in the blood smears?

      Your Answer:

      Correct Answer: Schistocytes

      Explanation:

      DIC, also known as consumptive coagulopathy, is a condition where the coagulation cascade is overactivated, leading to unchecked bleeding. This is due to the depletion of clotting mechanisms. Normally, clot formation and breakdown are balanced, with thrombin playing a key role in both processes. In DIC, patients may have prolonged coagulation times, thrombocytopenia, high levels of fibrin degradation products, elevated D-dimer levels, and microangiopathic pathology on peripheral smears. The excess fibrin strands in the intravascular circulation cause mechanical damage to red blood cells, resulting in schistocyte formation, thrombocytopenia, and consumption of clotting factors. Bite cells are abnormally shaped red blood cells with semicircular portions removed from the cell margin, seen in G6PD deficiency. Dacrocytes are teardrop-shaped cells seen in myelofibrosis and marrow disorders, while elliptocytes are red cells varying in shape from elongated to oval, seen in various disorders.

      Disseminated Intravascular Coagulation: A Condition of Simultaneous Coagulation and Haemorrhage

      Disseminated intravascular coagulation (DIC) is a medical condition characterized by simultaneous coagulation and haemorrhage. It is caused by the initial formation of thrombi that consume clotting factors and platelets, ultimately leading to bleeding. DIC can be caused by various factors such as infection, malignancy, trauma, liver disease, and obstetric complications.

      Clinically, bleeding is usually the dominant feature of DIC, accompanied by bruising, ischaemia, and organ failure. Blood tests can reveal prolonged clotting times, thrombocytopenia, decreased fibrinogen, and increased fibrinogen degradation products. The treatment of DIC involves addressing the underlying cause and providing supportive management.

      In summary, DIC is a serious medical condition that requires prompt diagnosis and management. It is important to identify the underlying cause and provide appropriate treatment to prevent further complications. With proper care and management, patients with DIC can recover and regain their health.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 89 - Tom, a 50-year-old man, visits his primary care physician to discuss his medications....

    Incorrect

    • Tom, a 50-year-old man, visits his primary care physician to discuss his medications. He was recently hospitalized for a deep vein thrombosis (DVT) and was prescribed dabigatran to prevent future occurrences. Can you explain how this anticoagulant works?

      The mechanism of action of dabigatran is its ability to inhibit thrombin, a key enzyme in the blood clotting process. By blocking thrombin, dabigatran prevents the formation of blood clots and reduces the risk of DVT and other thromboembolic events. Unlike traditional anticoagulants such as warfarin, dabigatran does not require regular monitoring and has fewer drug interactions. However, it may increase the risk of bleeding and should be used with caution in patients with renal impairment.

      Your Answer:

      Correct Answer: Directly inhibits thrombin

      Explanation:

      Dabigatran is a DOAC that directly inhibits thrombin, a clotting factor that converts fibrinogen to fibrin strands. This impairs clot formation and can be reversed with idarucizumab in severe bleeding.

      Tranexamic acid inhibits the activation of plasminogen, which prevents the breakdown of fibrin clots and increases clotting. It is commonly used in menorrhagia.

      Other DOAC medications, such as rivaroxaban, apixaban, and edoxaban, inhibit clotting factor Xa, which activates thrombin. These medications can be reversed with recombinant human factor Xa.

      Warfarin is a vitamin K antagonist that inhibits the synthesis of clotting factors II, VII, IX, and X, as well as natural anticoagulants protein C and S. It initially increases the risk of clotting, so patients must take heparin injections when first starting warfarin.

      Aspirin irreversibly inhibits COX, an enzyme that synthesizes thromboxanes, which promote platelet aggregation and vasoconstriction. By inhibiting thromboxane production, aspirin is effective in preventing myocardial infarction and stroke.

      Direct oral anticoagulants (DOACs) are medications used to prevent stroke in non-valvular atrial fibrillation (AF), as well as for the prevention and treatment of venous thromboembolism (VTE). To be prescribed DOACs for stroke prevention, patients must have certain risk factors, such as a prior stroke or transient ischaemic attack, age 75 or older, hypertension, diabetes mellitus, or heart failure. There are four DOACs available, each with a different mechanism of action and method of excretion. Dabigatran is a direct thrombin inhibitor, while rivaroxaban, apixaban, and edoxaban are direct factor Xa inhibitors. The majority of DOACs are excreted either through the kidneys or the liver, with the exception of apixaban and edoxaban, which are excreted through the feces. Reversal agents are available for dabigatran and rivaroxaban, but not for apixaban or edoxaban.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds
  • Question 90 - A 60-year-old male comes to you with complaints of fatigue and difficulty breathing...

    Incorrect

    • A 60-year-old male comes to you with complaints of fatigue and difficulty breathing for the past 2 months. During the physical examination, you observe that the patient is visibly jaundiced and the spleen is palpable. Upon conducting blood tests, the following results are obtained:

      Hb 98 g/l
      MCV 88 fl
      Direct Coombs test Pos

      Further testing is done to determine the antibody specificity, and the patient is diagnosed with warm autoimmune haemolytic anaemia. Which immunoglobulin is most likely responsible for mediating this condition?

      Your Answer:

      Correct Answer: IgG

      Explanation:

      Warm autoimmune haemolytic anaemia involves IgG-mediated red blood cell destruction at body temperature, while IgM-mediated haemolysis is precipitated by the cold and affects the hands and feet. Other immunoglobulins such as IgA and IgE may also be involved.

      Understanding Autoimmune Haemolytic Anaemia

      Autoimmune haemolytic anaemia (AIHA) is a condition where the body’s immune system attacks its own red blood cells, leading to anaemia. There are two types of AIHA: warm and cold. Warm AIHA is the most common type and is caused by an antibody (usually IgG) that causes haemolysis at body temperature. It tends to occur in the spleen and is often idiopathic, but can also be secondary to autoimmune diseases, neoplasia, or drugs. On the other hand, cold AIHA is caused by an IgM antibody that causes haemolysis at 4°C and is more commonly intravascular. It is associated with neoplasia and infections, and patients may experience symptoms of Raynaud’s and acrocynaosis.

      To diagnose AIHA, doctors look for general features of haemolytic anaemia, such as anaemia, reticulocytosis, low haptoglobin, raised lactate dehydrogenase (LDH) and indirect bilirubin, and spherocytes and reticulocytes on a blood film. A positive direct antiglobulin test (Coombs’ test) is specific for AIHA. Treatment for AIHA involves managing any underlying disorder and using steroids as first-line therapy, with rituximab as an option. However, patients with cold AIHA tend to respond less well to steroids.

      In summary, AIHA is a condition where the immune system attacks red blood cells, leading to anaemia. Warm and cold AIHA are the two types, with warm being more common and caused by an IgG antibody that causes haemolysis at body temperature, while cold is caused by an IgM antibody that causes haemolysis at 4°C and is associated with neoplasia and infections. Diagnosis involves looking for general features of haemolytic anaemia and a positive direct antiglobulin test. Treatment involves managing any underlying disorder and using steroids as first-line therapy.

    • This question is part of the following fields:

      • Haematology And Oncology
      0
      Seconds

SESSION STATS - PERFORMANCE PER SPECIALTY

Haematology And Oncology (3/9) 33%
Passmed