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Question 1
Correct
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A 56-year-old man who comes to see you concerned about prostate cancer. You perform a digital rectal exam which reveals an enlarged, firm and irregular prostate. What would be the best course of action for management?
Your Answer: Refer to urology as 2 week wait referral
Explanation:The appropriate way to address the patient is as a 2-week wait referral. According to the NICE Guidelines for suspected cancer referrals, if a hard, irregular prostate indicative of prostate carcinoma is detected during rectal examination, urgent referral is necessary. Along with the referral, the PSA test result should also be included. It is important to note that the PSA test is not always reliable in detecting prostate cancer, as around 1 in 50 men with fast-growing prostate cancer have a normal PSA level.
Prostate specific antigen (PSA) is an enzyme produced by both normal and cancerous prostate cells. It is commonly used as a marker for prostate cancer, but its effectiveness as a screening tool is still debated. The NHS Prostate Cancer Risk Management Programme (PCRMP) has released guidelines for handling requests for PSA testing in asymptomatic men. While a recent European trial showed a reduction in prostate cancer deaths, it also revealed a high risk of over-diagnosis and over-treatment. As a result, the National Screening Committee has decided not to introduce a screening programme, but rather allow men to make an informed decision. The PCRMP recommends age-adjusted upper limits for PSA levels, while NICE Clinical Knowledge Summaries suggest a lower threshold for referral. PSA levels can also be raised by factors such as benign prostatic hyperplasia, prostatitis, and urinary tract infections.
The specificity and sensitivity of PSA testing are poor, with a significant number of men with elevated PSA levels not having prostate cancer, and some with normal PSA levels having the disease. Various methods are used to add meaning to PSA levels, including age-adjusted upper limits and monitoring changes in PSA levels over time. It is also debated whether digital rectal examination causes a rise in PSA levels. It is important to note that PSA testing should be postponed after certain events, such as ejaculation or instrumentation of the urinary tract.
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This question is part of the following fields:
- Haematology/Oncology
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Question 2
Correct
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A 65-year-old man with a history of depression and lumbar spinal stenosis presents with a swollen and painful left calf. He is evaluated in the DVT clinic and a raised D-dimer is detected. A Doppler scan reveals a proximal deep vein thrombosis. Despite being active and feeling well, the patient has not undergone any recent surgeries or been immobile for an extended period. As a result, he is initiated on a direct oral anticoagulant. What is the optimal duration of treatment?
Your Answer: 6 months
Explanation:For provoked cases of venous thromboembolism, such as those following recent surgery, warfarin treatment is typically recommended for a duration of three months. However, for unprovoked cases, where the cause is unknown, a longer duration of six months is typically recommended.
NICE updated their guidelines on the investigation and management of venous thromboembolism (VTE) in 2020. The use of direct oral anticoagulants (DOACs) is recommended as first-line treatment for most people with VTE, including as interim anticoagulants before a definite diagnosis is made. Routine cancer screening is no longer recommended following a VTE diagnosis. The cornerstone of VTE management is anticoagulant therapy, with DOACs being the preferred choice. All patients should have anticoagulation for at least 3 months, with the length of anticoagulation being determined by whether the VTE was provoked or unprovoked.
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This question is part of the following fields:
- Haematology/Oncology
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Question 3
Correct
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A 19-year-old man is being examined for excessive bleeding after a tooth extraction. The test results show:
Platelet count: 173 * 109/l
Prothrombin time (PT): 12.9 seconds
Activated partial thromboplastin time (APTT): 84 seconds
Which clotting factor is he most likely deficient in?Your Answer: Factor VIII
Explanation:Understanding Haemophilia
Haemophilia is a genetic disorder that affects blood coagulation. It is caused by a deficiency in either factor VIII (Haemophilia A) or factor IX (Haemophilia B), which are essential for blood clotting. Although it is an X-linked recessive disorder, up to 30% of patients have no family history of the condition.
The symptoms of haemophilia include haemoarthroses, haematomas, and prolonged bleeding after surgery or trauma. Blood tests can confirm the diagnosis, with a prolonged APTT being a common finding. However, the bleeding time, thrombin time, and prothrombin time are usually normal.
Treatment for haemophilia involves replacing the missing clotting factor through regular infusions. However, up to 10-15% of patients with haemophilia A may develop antibodies to factor VIII treatment, which can make it less effective.
In summary, haemophilia is a genetic disorder that affects blood coagulation and can cause symptoms such as haemoarthroses and prolonged bleeding. Diagnosis is confirmed through blood tests, and treatment involves replacing the missing clotting factor.
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This question is part of the following fields:
- Haematology/Oncology
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Question 4
Incorrect
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A 50-year-old woman who recently finished a round of chemotherapy reports experiencing tingling sensations and difficulty using her hands. Additionally, she has noticed hesitancy in her urinary function. Which specific cytotoxic medication is the likely culprit for these symptoms?
Your Answer: Bleomycin
Correct Answer: Vincristine
Explanation:Peripheral neuropathy is a known side effect of Vincristine. Additionally, bladder atony may cause urinary hesitancy.
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 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, degrade preformed DNA and can lead to lung fibrosis. Anthracyclines, such as doxorubicin, stabilize the DNA-topoisomerase II complex and inhibit DNA and RNA synthesis, but can also cause cardiomyopathy. Antimetabolites, like methotrexate, inhibit dihydrofolate reductase and thymidylate synthesis, leading to myelosuppression, mucositis, liver fibrosis, and lung fibrosis. Fluorouracil (5-FU) is a pyrimidine analogue that induces cell cycle arrest and apoptosis by blocking thymidylate synthase, but can also cause myelosuppression, mucositis, and dermatitis. Cytarabine is a pyrimidine antagonist that interferes with DNA synthesis specifically at the S-phase of the cell cycle and inhibits DNA polymerase, but can also cause myelosuppression and ataxia. Drugs that act on microtubules, like vincristine and vinblastine, inhibit the formation of microtubules and can cause peripheral neuropathy, paralytic ileus, and myelosuppression. Docetaxel prevents microtubule depolymerisation and disassembly, decreasing free tubulin, but can also cause neutropaenia. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I which prevents relaxation of supercoiled DNA, but can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin, cause cross-linking in DNA and can lead to ototoxicity, peripheral neuropathy, and hypomagnesaemia. Hydroxyurea (hydroxycarbamide) inhibits ribonucleotide reductase, decreasing DNA synthesis, but can also cause myelosuppression.
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This question is part of the following fields:
- Haematology/Oncology
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Question 5
Incorrect
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A 30-year-old woman comes to her General Practitioner complaining of fatigue and sudden shortness of breath. An urgent full blood count is performed and the patient is diagnosed with acute lymphoblastic leukemia (ALL).
What is the most probable first-line treatment option for this patient's leukemia?Your Answer: Blood transfusion
Correct Answer: Chemotherapy
Explanation:Treatment Options for Acute Leukaemia: Chemotherapy, Stem-Cell Transplant, Blood Transfusion, Intravenous Immunoglobulins, and Radiotherapy
Acute leukaemias, such as acute lymphoblastic leukaemia (ALL), are characterized by an increase in primitive undifferentiated blast cells in the bone marrow and blood, leading to marrow failure. The traditional treatment for ALL involves four components: induction, consolidation, maintenance, and central nervous system (CNS) prophylaxis. The first-line therapy for ALL is combination chemotherapy, which aims to eradicate the blast cells. Once remission is induced, maintenance chemotherapy is given to eliminate the disease that cannot be detected under the microscope.
Stem-cell transplants can be used to treat ALL once remission is induced using chemotherapy. This treatment can be allogeneic (the patient receives stem cells from a matched or partially mismatched related or unrelated donor) or autologous (the patient receives their own stem cells). The goal of a stem-cell transplant is to restore the body’s ability to produce normal blood cells. While it can be a curative treatment for patients with this disease, it is not used as a first-line treatment.
Blood transfusions are not a treatment for acute leukaemia, but they may be necessary to treat anaemia or platelet deficiency. Patients with leukaemia are at risk of graft-versus-host disease, so they are typically given irradiated blood components. Intravenous immunoglobulins are not used to treat acute leukaemia but may be used prophylactically against infection in patients with hypogammaglobulinaemia due to cancer treatment.
Radiotherapy is not a first-line treatment for acute leukaemia, but it may be used to treat the brain or spinal cord if the disease has spread there. Total body radiotherapy may also be used before a stem-cell transplant to suppress the immune system and reduce the risk of transplant rejection.
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This question is part of the following fields:
- Haematology/Oncology
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Question 6
Correct
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A 12-year-old boy presents acutely with petechiae on his legs, severe abdominal pain, bloody faeces, haematuria and painful joint swelling. The haematology laboratory results are normal.
Which of the following is the most likely diagnosis?
Your Answer: Henoch–Schönlein purpura
Explanation:Common Pediatric Diseases: Symptoms and Management
Henoch–Schönlein purpura (HSP), Acute lymphocytic leukaemia, Alport’s syndrome, and Juvenile rheumatoid arthritis (JRA) are some of the common pediatric diseases that require prompt diagnosis and management. HSP is an autoimmune hypersensitivity vasculitis that often affects children and is associated with IgA dominant immune complexes. ALL is a type of cancer that affects the blood and bone marrow, causing symptoms such as fatigue, fever, and joint pain. Alport’s syndrome is a hereditary nephritis that can lead to chronic kidney disease, hearing loss, and ocular abnormalities. JRA is characterized by swollen joints, fever, and joint pain. Prompt diagnosis and management are crucial in these diseases, which may require supportive treatment, pain relief, and monitoring for potential complications.
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This question is part of the following fields:
- Haematology/Oncology
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Question 7
Incorrect
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A 68-year-old woman is referred with fatigue. Her primary care physician observes that she has jaundice and suspects liver disease. She also presents with angular cheilitis. She has a history of taking steroid inhalers for asthma, but no other significant medical history. A blood smear shows signs of megaloblastic anemia, and her serum bilirubin level is elevated, but her other laboratory results are normal. There are no indications of gastrointestinal (GI) issues.
What is the most appropriate diagnosis for this clinical presentation?Your Answer: Crohn's disease
Correct Answer: Pernicious anaemia
Explanation:Differential Diagnosis of Anaemia: Understanding the Causes
Anaemia is a common condition that can be caused by a variety of factors. Here, we will discuss some of the possible causes of anaemia and their corresponding laboratory findings.
Pernicious Anaemia: This type of anaemia is caused by a deficiency in vitamin B12 due to impaired intrinsic factor (IF) production. It is usually seen in adults aged 40-70 years and is characterized by megaloblastic changes in rapidly dividing cells. Anti-parietal cell antibodies are present in 90% of patients with pernicious anaemia. The Schilling test is useful in confirming the absence of IF. Treatment involves parenteral administration of cyanocobalamin or hydroxycobalamin.
Chronic Myeloid Leukaemia: CML is a myeloproliferative disorder that results in increased proliferation of granulocytic cells. Symptoms include fatigue, anorexia, weight loss, and hepatosplenomegaly. Mild to moderate anaemia is usually normochromic and normocytic. Diagnosis is based on histopathological findings in the peripheral blood and Philadelphia chromosome in bone marrow cells.
Iron Deficiency Anaemia: This type of anaemia is primarily a laboratory diagnosis and is characterized by microcytic and hypochromic erythropoiesis. It is caused by chronic iron deficiency and can be due to multiple causes, including chronic inflammation, iron malabsorption, chronic blood loss, and malabsorption of vitamin B12 or folate.
Crohn’s Disease: This chronic inflammatory process can affect any part of the GI tract and can cause anaemia due to chronic inflammation, iron malabsorption, chronic blood loss, and malabsorption of vitamin B12 or folate. However, the lack of GI symptoms in the clinical scenario provided is not consistent with a history of inflammatory bowel disease.
Autoimmune Hepatitis: This chronic disease is characterized by continuing hepatocellular inflammation and necrosis, with a tendency to progress to cirrhosis. Elevated serum aminotransferase levels are present in 100% of patients at initial presentation. Anaemia, if present, is usually normochromic. However, this clinical picture and laboratory findings are not consistent with the scenario given.
In conclusion, understanding the different causes of anaemia and their corresponding laboratory findings is crucial in making an accurate diagnosis and providing appropriate treatment.
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This question is part of the following fields:
- Haematology/Oncology
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Question 8
Incorrect
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A 64-year-old woman presents to the clinic after undergoing a lumpectomy for a cancerous lump in her left breast. The histopathology report indicates that the tissue was oestrogen receptor-positive, and the patient is offered anastrozole therapy as adjuvant treatment. She has no significant medical history and is not currently taking any medications. The patient is eager to begin the recommended adjuvant therapy. What diagnostic tests should be conducted before initiating treatment?
Your Answer: Liver function blood tests
Correct Answer: DEXA scan
Explanation:Anastrozole, an aromatase inhibitor, is a recommended adjuvant therapy for patients with oestrogen positive cancer (ER+). However, it may lead to osteoporosis as an adverse effect. Therefore, NICE recommends performing a DEXA scan before initiating therapy. A clotting screen is not necessary before starting anastrozole, as it does not cause coagulopathies. ECGs are not required either, as cardiac changes and arrhythmias are not associated with this medication. Unlike certain medications like statins, anastrozole is not known to commonly affect lipid profiles, so it does not need to be monitored. Liver function tests are also not routinely assessed before starting anastrozole, as it is not known to affect liver function. However, it would have been appropriate to send a clotting screen before the patient’s lumpectomy.
Anti-oestrogen drugs are used in the management of oestrogen receptor-positive breast cancer. Selective oEstrogen Receptor Modulators (SERM) such as Tamoxifen act as an oestrogen receptor antagonist and partial agonist. However, Tamoxifen can cause adverse effects such as menstrual disturbance, hot flushes, venous thromboembolism, and endometrial cancer. On the other hand, aromatase inhibitors like Anastrozole and Letrozole reduce peripheral oestrogen synthesis, which is important in postmenopausal women. Anastrozole is used for ER +ve breast cancer in this group. However, aromatase inhibitors can cause adverse effects such as osteoporosis, hot flushes, arthralgia, myalgia, and insomnia. NICE recommends a DEXA scan when initiating a patient on aromatase inhibitors for breast cancer.
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This question is part of the following fields:
- Haematology/Oncology
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Question 9
Correct
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A 5-year-old boy with sickle cell anaemia complains of abdominal pain. During the physical examination, the doctor observes splenomegaly and signs of anaemia. What is the probable diagnosis?
Your Answer: Sequestration crisis
Explanation:In a sequestration crisis, the sickle cells lead to significant enlargement of the spleen, which causes abdominal pain as seen in this case. This is more prevalent in early childhood as repeated sequestration and infarction of the spleen during childhood can eventually lead to an auto-splenectomy. A sequestration crisis can result in severe anemia, noticeable pallor, and cardiovascular collapse due to the loss of effective circulating volume.
Sickle cell anaemia is a condition that involves periods of good health with intermittent crises. There are several types of crises that can occur, including thrombotic or painful crises, sequestration, acute chest syndrome, aplastic, and haemolytic. Thrombotic crises, also known as painful crises or vaso-occlusive crises, are triggered by factors such as infection, dehydration, and deoxygenation. These crises are diagnosed clinically and can result in infarcts in various organs, including the bones, lungs, spleen, and brain.
Sequestration crises occur when sickling occurs within organs such as the spleen or lungs, leading to pooling of blood and worsening of anaemia. This type of crisis is associated with an increased reticulocyte count. Acute chest syndrome is caused by vaso-occlusion within the pulmonary microvasculature, resulting in infarction in the lung parenchyma. Symptoms include dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, and low pO2. Management involves pain relief, respiratory support, antibiotics, and transfusion.
Aplastic crises are caused by infection with parvovirus and result in a sudden fall in haemoglobin. Bone marrow suppression leads to a reduced reticulocyte count. Haemolytic crises are rare and involve a fall in haemoglobin due to an increased rate of haemolysis. It is important to recognise and manage these crises promptly, as they can lead to serious complications and even death.
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This question is part of the following fields:
- Haematology/Oncology
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Question 10
Incorrect
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A 30-year-old male presents with progressive weakness and fatigue over the past year. He reports difficulty achieving and maintaining an erection with his new partner, which is a new symptom for him. Laboratory tests reveal elevated ferritin levels. The patient is started on a treatment plan that involves regular phlebotomies.
What is the most effective way to monitor the patient's response to treatment?Your Answer: Serum iron and transferrin saturations
Correct Answer: Ferritin and transferrin saturation
Explanation:To monitor treatment in haemochromatosis, the most effective combination of iron tests is ferritin and transferrin saturation. These tests can track the response to treatment by measuring total iron stores and the amount of serum iron bound to proteins in the blood. However, serum transferrin and serum iron are not reliable indicators of treatment response as they fluctuate throughout the day and are affected by diet and phlebotomies. Therefore, using ferritin and serum transferrin or serum iron would not be the most useful combination for monitoring haemochromatosis. Similarly, using serum iron and serum transferrin together would not provide any insight into treatment monitoring. The most appropriate and effective combination is ferritin and transferrin saturation.
Understanding Haemochromatosis: Investigation and Management
Haemochromatosis is a genetic disorder that causes iron accumulation in the body due to mutations in the HFE gene on both copies of chromosome 6. The best investigation to screen for haemochromatosis is still a topic of debate. For the general population, transferrin saturation is considered the most useful marker, while genetic testing for HFE mutation is recommended for testing family members. Diagnostic tests include molecular genetic testing for the C282Y and H63D mutations and liver biopsy with Perl’s stain. A typical iron study profile in a patient with haemochromatosis includes high transferrin saturation, raised ferritin and iron, and low TIBC.
The first-line treatment for haemochromatosis is venesection, which involves removing blood from the body to reduce iron levels. Transferrin saturation should be kept below 50%, and the serum ferritin concentration should be below 50 ug/l to monitor the adequacy of venesection. If venesection is not effective, desferrioxamine may be used as a second-line treatment. Joint x-rays may show chondrocalcinosis, which is a characteristic feature of haemochromatosis. It is important to note that there are rare cases of families with classic features of genetic haemochromatosis but no mutation in the HFE gene.
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This question is part of the following fields:
- Haematology/Oncology
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Question 11
Correct
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A 32-year-old man is brought to the emergency department by his physician due to bone pain, weakness, and splenomegaly. He has been experiencing increasing fatigue and weakness while performing his daily tasks over the past few weeks. During the examination, he appears extremely pale and has petechiae on his lower limbs. After a bone marrow sample is taken, he is diagnosed with acute promyelocytic leukemia (APML). What is the most probable finding on his blood film?
Your Answer: Auer rods
Explanation:A finding of Auer rods on a blood film strongly indicates the presence of acute promyelocytic leukemia.
The correct answer is ‘Auer rods’. This patient has been diagnosed with APML, and the most common finding on a blood film associated with this is Auer rods. These are needle-like structures that are large, pink or red stained and can be seen within the cytoplasm of myeloid blast cells.
‘Tear-drop’ poikilocytes are typically found in myelofibrosis, which is characterized by flat, elongated red blood cells that resemble a tear-drop in shape. This occurs due to the squeezing of cells through fibrotic tissue in bone marrow in myelofibrotic disorders.
Smear cells are usually seen in chronic lymphocytic leukemia (CLL), which are remnants of cells that lack identifiable plasma membrane or nuclear structure.
Spherocytes are generally found in hereditary spherocytosis or autoimmune hemolytic anemia, which are red blood cells that are sphere-shaped and more fragile than normal red blood cells due to abnormalities in the red cell membrane.
Acute myeloid leukaemia is a common form of acute leukaemia in adults that may occur as a primary disease or following a myeloproliferative disorder. Symptoms are related to bone marrow failure and include anaemia, neutropenia, thrombocytopenia, splenomegaly, and bone pain. Poor prognostic features include age over 60, >20% blasts after first course of chemo, and deletions of chromosome 5 or 7. Acute promyelocytic leukaemia M3 is associated with t(15;17) and has a good prognosis. The French-American-British classification system includes seven subtypes.
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This question is part of the following fields:
- Haematology/Oncology
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Question 12
Correct
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A 4-year-old girl presents to the Emergency Department with haemarthrosis in the knee and elbow after a trivial injury. She is otherwise fit and well. The patient’s mother suffers from Christmas disease.
What investigation should be organised to confirm this diagnosis in this patient?Your Answer: Factor IX activity level
Explanation:Diagnostic Tests for Haemophilia and Related Conditions
Haemophilia is a genetic disorder that affects the blood’s ability to clot properly, leading to spontaneous or excessive bleeding. To diagnose haemophilia and related conditions, several diagnostic tests are available.
Factor IX Activity Level: Lack of factor IX leads to Christmas disease, the second most common type of haemophilia. To diagnose haemophilia, the levels of clotting factor present in the blood must be measured.
Platelet Count: The platelet count is usually normal in patients with haemophilia. Reduced platelets would suggest an alternative diagnosis. However, it is still important to check the platelet count to exclude thrombocytopenia as a cause of unexplained bleeding.
Activated Partial Thromboplastin Time (aPTT): Blood tests in haemophilia usually demonstrate a prolonged aPTT. However, patients with moderate disease can still have a normal aPTT if their factor activity level is > 15%.
Urinalysis: Urinalysis in patients with haemophilia may demonstrate the presence of haematuria, but by itself is not diagnostic of the condition.
von Willebrand Factor Antigen: The plasma von Willebrand factor antigen is normal in individuals with haemophilia. Reduced von Willebrand factor suggests the possibility of von Willebrand disease (VWD).
In conclusion, a combination of these diagnostic tests is necessary to diagnose haemophilia and related conditions accurately.
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This question is part of the following fields:
- Haematology/Oncology
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Question 13
Correct
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A 30-year-old woman has just been diagnosed with Hodgkin's lymphoma and has started her first day of chemotherapy. However, she has been feeling unwell since then and her U&E results show abnormalities such as hyperkalaemia, hyperphosphataemia, and hypocalcaemia. What prophylaxis is used to prevent the complication that this woman has experienced?
Your Answer: Allopurinol
Explanation:Tumour lysis syndrome is characterized by high levels of potassium and phosphate, as well as low levels of calcium. This is evident in the case history of a lymphoma patient who has started chemotherapy. Allopurinol is the most commonly used prophylaxis, with rasburicase as an alternative. Diuretics are not recommended due to the potential to exacerbate acute kidney injury. It is important to note that steroids and radiotherapy are not common causes of tumour lysis syndrome.
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. Although it can happen 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. Rasburicase is a recombinant version of urate oxidase, an enzyme that metabolizes uric acid to allantoin, which is more easily excreted by the kidneys. Patients in lower-risk groups should be given oral allopurinol during chemotherapy cycles to avoid the condition. However, rasburicase and allopurinol should not be given together in the management of tumour lysis syndrome as this reduces the effect of rasburicase.
TLS occurs when tumour cells break down and release chemicals into the body, leading to high levels of potassium and phosphate and a low level of calcium. It should be suspected in any patient presenting with an acute kidney injury in the presence of high phosphate and uric acid levels. From 2004, TLS has been graded using the Cairo-Bishop scoring system, which considers abnormality in two or more of the following within three days before or seven days after chemotherapy: uric acid, potassium, phosphate, and calcium. Clinical tumour lysis syndrome is when laboratory tumour lysis syndrome is present along with increased serum creatinine, cardiac arrhythmia or sudden death, or seizure.
In summary, understanding tumour lysis syndrome is critical in the treatment of high-grade lymphomas and leukaemias. Prophylactic medication can be given to prevent the potentially deadly effects of tumour cell lysis, and the Cairo-Bishop scoring system can be used to grade the severity of the condition. Early detection and management of TLS can improve patient outcomes and prevent complications.
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This question is part of the following fields:
- Haematology/Oncology
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Question 14
Correct
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A 30-year-old man presents approximately 2 weeks after a viral illness with petechiae, easy bruising, gum bleeding and lesions of the oral mucosa and tongue. He has no personal or family history of note and is not taking any medication. Physical examination is normal, except for petechiae, bruising and oral lesions. An image of the patient's tongue is shown below. What is the most appropriate initial investigation/management option for this clinical scenario and image?
Your Answer: Full blood count
Explanation:Diagnosis and Investigation of Immune Thrombocytopenic Purpura (ITP)
Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by antiplatelet antibodies and immune-mediated platelet destruction, leading to a decrease in peripheral platelet count and an increased risk of severe bleeding. The following are the recommended diagnostic and investigative measures for ITP:
Full Blood Count: An urgent first-line investigation with a full blood count is essential to confirm platelet count, which is the hallmark of ITP. Anaemia and/or neutropenia may indicate other diseases. On peripheral blood smear, the morphology of red blood cells and leukocytes is normal, while the morphology of platelets is usually normal, with varying numbers of large platelets.
Surgical Correction: Surgical management is not the first-line intervention for ITP and is reserved for later use in patients with acute ITP, for whom splenectomy usually results in rapid, complete, and life-long clinical remission. Its results in patients with chronic ITP are typically less predictable.
Aspiration for Cytology: Further investigations, such as bone marrow aspiration, could be considered for later use in patients with ITP, such as adults older than 60 years, to exclude myelodysplastic syndrome or leukemia.
Excision Biopsy: Further investigations, such as a biopsy, could be considered further down the line, such as in adults older than 60 years, to exclude myelodysplastic syndrome or leukemia.
None: If a patient presents with purpura/petechiae on the tongue and buccal mucosa, urgent haematological referral is warranted, and not undertaking any further investigations would be inappropriate.
Diagnosis and Investigation of Immune Thrombocytopenic Purpura (ITP)
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This question is part of the following fields:
- Haematology/Oncology
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Question 15
Correct
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An 85-year-old man complains of increasing back pain and overall weakness. An X-ray of his back reveals multiple vertebral collapses and lytic lesions.
What is the most probable diagnosis?Your Answer: Multiple myeloma
Explanation:Understanding Multiple Myeloma: A Malignant Disease of Plasma Cells
Multiple myeloma is a malignant disease of plasma cells that commonly affects adults aged over 60 years. It is characterized by the production of paraproteinaemia in the blood, which can affect multiple organs including the blood, bone, kidneys, and immune system. The most common symptoms of multiple myeloma include unexplained bone pain, hypercalcaemia, weight loss, hyperviscosity symptoms, spinal cord compression, and fever.
On the other hand, osteoporosis is a condition characterized by low bone mass and deterioration of the structure of the bone tissue, resulting in bone fragility and susceptibility to fracture. Multiple vertebral collapses and lytic lesions are more sinister in nature and are commonly associated with multiple myeloma.
Osteosarcoma, bone metastases, and chronic myeloblastic leukaemia (CML) are not related to multiple myeloma. Osteosarcoma is characterized by cortical destruction and the presence of a soft-tissue mass, while bone metastases usually lead to destruction of the cortex on plain radiographs. Symptoms of CML include fatigue, night sweats, weight loss, abdominal fullness, and left upper quadrant pain.
In summary, understanding the symptoms and characteristics of multiple myeloma is crucial in diagnosing and treating this malignant disease of plasma cells.
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This question is part of the following fields:
- Haematology/Oncology
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Question 16
Correct
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A 70-year-old retired car mechanic who is a smoker is diagnosed with lung cancer. Some fifteen years ago he was treated with surgery and adjuvant chemotherapy for bladder cancer. His brother had oesophageal cancer aged 66 and another brother had prostate cancer in his 70s. His 55-year-old daughter was recently diagnosed with breast cancer.
Which of the following is the most likely explanation for the cancers in your patient?Your Answer: Exposure to tobacco smoke
Explanation:Understanding the Possible Causes of Multiple Cancers in an Individual
It is not uncommon for an individual to develop two different types of cancer over their lifetime. In a case where a man has been diagnosed with both bladder and lung cancer, the possible causes need to be explored.
Exposure to tobacco smoke is the most likely cause of both cancers in this case. Smoking is responsible for 85% of lung cancers and is also the biggest risk factor for developing bladder cancer.
Exposure to asbestos or aniline dyes are not likely causes of both cancers. While asbestos exposure is associated with mesothelioma, it is not linked to bladder cancer. Similarly, aniline dyes increase the risk of bladder cancer but not lung cancer.
A hereditary cancer predisposition syndrome is also an unlikely cause in this case. While it is possible for an individual to have a genetic predisposition to developing multiple cancers, there are no specific patterns in the family history that suggest this.
Finally, the second tumor is not a late complication of chemotherapy. While previous cancer treatment could potentially increase the risk of developing lung cancer, smoking is still the most likely cause in this case.
In conclusion, exposure to tobacco smoke is the most likely cause of both bladder and lung cancer in this individual. It is important to identify the possible causes of multiple cancers in an individual to ensure appropriate treatment and management.
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This question is part of the following fields:
- Haematology/Oncology
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Question 17
Correct
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A 68-year-old woman presents to her General Practitioner to discuss some recent blood tests which were taken for tri-monthly monitoring of her methotrexate. She has rheumatoid arthritis (RA) and takes methotrexate, folic acid and co-codamol.
Investigations:
Investigation Result Normal value
Haemoglobin 91 g/l 115–165 g/l
White cell count (WCC) 5.2 × 109/l 4.0–11.0 × 109/l
Platelets 228 × 109/l 150–400 × 109/l
Neutrophils 5.4 × 109/l 2.0–7.5 × 109/l
Mean corpuscular volume (MCV) 96 fl 85–105 fl
Mean corpuscular haemoglobin (MCH) 29 pg 27–32 pg
Sodium 138 mmol/l 135–145 mmol/l
Potassium 4.1 mmol/l 3.5–5.3 mmol/l
Urea 3.2 mmol/l 2.5–7.5 mmol/l
Creatinine 68 µmol/l 53–100 µmol/l
Estimated glomerular filtration rate > 90 ml/min per 1.73m2 > 90 ml/min per 1.73m2
What is the most likely cause of this patient’s anaemia?
Your Answer: Anaemia of chronic disease
Explanation:Understanding the Causes of Normocytic Anaemia in a Patient with Rheumatoid Arthritis
The patient in question has been diagnosed with normocytic anaemia, which is characterized by normal MCV and MCH results. There are several potential causes of this type of anaemia, including renal failure, anaemia of chronic disease, and mixed iron and vitamin B12 or folate deficiency. However, given that the patient has rheumatoid arthritis (RA) and normal renal function, the most likely cause of her anaemia is a chronic disease. This is thought to be the result of chronic inflammation associated with diseases such as RA.
One potential complication of RA is Felty syndrome, which is characterized by a triad of conditions: RA, splenomegaly, and neutropenia. However, this patient has a normal WCC and neutrophil count, which rules out this diagnosis.
Vitamin B12 deficiency can also cause anaemia, but it typically results in macrocytic anaemia characterized by a raised MCV. In contrast, this patient has a normal MCV. Vitamin B12 deficiency is typically treated with oral supplements, unless intrinsic antibodies are present, in which case intramuscular B12 is needed.
Folate deficiency can also drive macrocytic anaemia, but this patient demonstrates normocytic anaemia. Methotrexate, which is commonly used to treat RA, is a folate antagonist, which is why the patient is also taking folic acid supplements to reduce the risk of developing folate deficiency.
Iron deficiency is another potential cause of anaemia, but it typically results in microcytic hypochromic anaemia characterized by low MCV and MCH. In contrast, this patient has normal MCV and MCH results. A combination of iron and vitamin B12 or folate deficiencies may result in normocytic anaemia, as can acute blood loss.
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This question is part of the following fields:
- Haematology/Oncology
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Question 18
Correct
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A 78-year-old man with known metastatic prostate cancer presents to his General Practitioner with generalised pain. He states that he has always had aches and pains from old age, but that this is different: he can feel the pain particularly in his back at night.
What is the most likely site to be involved in bone metastasis in this patient?Your Answer: Spine
Explanation:Common Sites of Metastatic Spread in Bone
Metastatic spread to the bone is a common occurrence in many types of cancer. The following are some of the most common sites of metastases in bone:
Spine: The spine is the most common site for bony metastases, with spread often found from a range of solid and haematological cancers, as well as infectious diseases such as tuberculosis.
Ribs: While breast cancer is known to spread to the ribs, this is not the case for many other cancers.
Pelvis: The pelvis is a prevalent site of metastatic spread occurring mostly from the prostate, breast, kidney, lung, and thyroid cancer.
Skull: Skull metastases are seen in 15-25% of all cancer patients and tend to include those from the breast, lungs, prostate, and thyroid, as well as melanoma.
Long bones: Localised bone pain is a red flag for metastatic spread to any of the long bones such as the femur and humerus. Breast, prostate, renal, thyroid, and lung cancers frequently metastasize to these areas.
Overall, understanding the common sites of metastatic spread in bone can help with early detection and treatment of cancer.
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This question is part of the following fields:
- Haematology/Oncology
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Question 19
Correct
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A 32-year-old woman presents with heavy menstrual bleeding and a haemoglobin level of 102 g/L. Iron studies are ordered to investigate for iron-deficiency anaemia. What result would support this diagnosis?
Your Answer: ↓ Ferritin, ↑ total iron-binding capacity, ↓ serum iron, ↓ transferrin saturation
Explanation:In cases of iron-deficiency anemia, it is common for both the total iron-binding capacity (TIBC) and transferrin levels to be elevated. However, it should be noted that the transferrin saturation level is typically decreased.
Iron deficiency anaemia is caused by a lack of iron, which is needed to make haemoglobin in red blood cells. It is the most common type of anaemia worldwide, with preschool-age children having the highest prevalence. Causes include excessive blood loss, inadequate dietary intake, poor intestinal absorption, and increased iron requirements. Symptoms include fatigue, shortness of breath, palpitations, and nail changes. Diagnosis is made through a full blood count and serum ferritin test. Treatment involves identifying and managing the underlying cause, as well as taking oral iron supplements and consuming an iron-rich diet.
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This question is part of the following fields:
- Haematology/Oncology
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Question 20
Correct
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A 14-year-old girl is referred to haematology due to heavy and prolonged periods that have not responded well to tranexamic acid and the combined oral contraceptive pill. Her blood tests reveal Hb of 10.3 g/dl, Plt of 239 * 109/l, WBC of 6.5 * 109/l, PT of 12.9 secs, and APTT of 37 secs. What is the probable diagnosis?
Your Answer: Von Willebrand's disease
Explanation:Understanding Von Willebrand’s Disease
Von Willebrand’s disease is a common inherited bleeding disorder that is usually passed down in an autosomal dominant manner. It behaves like a platelet disorder, with symptoms such as nosebleeds and heavy menstrual bleeding being common, while joint and muscle bleeding are rare. The disease is caused by a deficiency or abnormality in von Willebrand factor, a large glycoprotein that promotes platelet adhesion to damaged endothelium and acts as a carrier molecule for factor VIII.
There are three types of Von Willebrand’s disease. Type 1 is the most common and is characterized by a partial reduction in von Willebrand factor. Type 2 is further divided into four subtypes, each with a different abnormality in the von Willebrand factor. Type 3 is the most severe form and is caused by a total lack of von Willebrand factor, inherited in an autosomal recessive manner.
Diagnosis of Von Willebrand’s disease involves tests such as a prolonged bleeding time, APTT, factor VIII levels, and platelet aggregation with ristocetin. Management options include tranexamic acid for mild bleeding, desmopressin to raise levels of von Willebrand factor, and factor VIII concentrate. While there is no clear correlation between symptomatic presentation and type of Von Willebrand’s disease, common themes among patients include excessive mucocutaneous bleeding, bruising in the absence of trauma, and menorrhagia in females.
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This question is part of the following fields:
- Haematology/Oncology
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