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  • Question 1 - A 66-year-old female presents to the Emergency Department with a nose bleed. She...

    Incorrect

    • A 66-year-old female presents to the Emergency Department with a nose bleed. She says that she has been having frequent episodes of nose bleeds over the past four months, along with increasing fatigue and some weight loss. On examination, she has a diffuse petechial rash and hypertrophy of the gingiva.

      Which one of the following conditions is this patient most likely to have?

      Your Answer: Multiple myeloma

      Correct Answer: Acute myeloid leukaemia (AML)

      Explanation:

      The history of nosebleeds and fatigue, and gingival hyperplasia presents a typical picture of acute myeloid leukaemia. Leukemic infiltrates within the gingiva cause hypertrophy and distinguish this condition from other types of leukaemia. The fatigue is secondary to anaemia, while the nosebleeds are caused by thrombocytopenia secondary to leukemic infiltration of bone marrow. Patients may also report frequent infections secondary to neutropenia and hepatosplenomegaly.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      1
      Seconds
  • Question 2 - How does abciximab mediate its antiplatelet effect: ...

    Incorrect

    • How does abciximab mediate its antiplatelet effect:

      Your Answer:

      Correct Answer: It is a GPIIb/IIIa inhibitor.

      Explanation:

      Abciximab, eptifibatide and tirofiban are GPIIb/IIIa inhibitors, inhibiting platelet aggregation by preventing the binding of fibrinogen, von Willebrand factor and other adhesive molecules.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      6
      Seconds
  • Question 3 - A 20-year-old male who is a known patient of sickle cell disease presents...

    Incorrect

    • A 20-year-old male who is a known patient of sickle cell disease presents to the Emergency Room with a sustained erection that is extremely painful.

      Out of the following clinical syndromes, which one does this patient most likely have?

      Your Answer:

      Correct Answer: Priapism

      Explanation:

      Sickling of red blood cells can lead to several different clinical syndromes. If the sickling occurs in the corpora cavernosa, it can lead to a sustained, painful erection of the penis, referred to as priapism. One of the complications is long-term impotence. It is important to seek a urological opinion immediately in this case, but in the interim, treat with perineal ice packs and walk up and down the stairs.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 4 - Detection of urinary Bence-Jones protein would be most suggestive of which of the...

    Incorrect

    • Detection of urinary Bence-Jones protein would be most suggestive of which of the following malignancies:

      Your Answer:

      Correct Answer: Multiple myeloma

      Explanation:

      Bence Jones proteins are monoclonal immunoglobulin light chains found in urine (and plasma) of some patients with myeloma. Because of their relatively small size, light chains are readily excreted into the urine. The presence of significant amounts of Bence Jones proteins in urine is indicative of malignant B cell proliferation.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 5 - A six-year-old boy presents with coryzal symptoms that have persisted for more than...

    Incorrect

    • A six-year-old boy presents with coryzal symptoms that have persisted for more than two weeks. He was born and raised in the Middle East. His mother claims he has been tired and has complained of various 'aches and pains.' On examination, you find splenomegaly and enlarged cervical lymph nodes. His legs and arms are covered in petechiae.

      In this case, what is the most likely diagnosis?

      Your Answer:

      Correct Answer: Acute lymphoblastic leukaemia (ALL)

      Explanation:

      ALL is the most common leukaemia in children, with a peak incidence between the ages of 2 and 5.
      ALL has a wide range of clinical symptoms, but many children present with an acute illness that resembles coryza or a viral infection. ALL also has the following features:
      Weakness and sluggishness all over
      Muscle, joint, and bone pain that isn’t specific
      Anaemia
      Petechiae and unexplained bruising
      Oedema
      Lymphadenopathy
      Hepatosplenomegaly

      The following are typical features of a full blood count in patients with ALL:
      Anaemia (normocytic or macrocytic)
      Leukopenia affects about half of the patients (WCC 4 x 109/l).
      Around 25% of patients have leucocytosis (WCC > 10 x 109/l).
      Around 25% of patients have hyperleukocytosis (WCC > 50 x 109/l).
      Thrombocytopaenia

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 6 - Regarding iron handling, which of the following statements is CORRECT: ...

    Incorrect

    • Regarding iron handling, which of the following statements is CORRECT:

      Your Answer:

      Correct Answer: Iron is taken across the enterocyte apical membrane by the divalent metal transporter (DMT1).

      Explanation:

      Dietary iron may be in the form of haem or non-haem iron. Haem iron is degraded after absorption through the cell surface to release Fe2+. Most non-haem iron is in the form Fe3+, which is reduced at the luminal surface to the more soluble Fe2+, facilitated by hydrochloric acid in gastric secretions (and enhanced by ascorbic acid). Fe2+is taken across the enterocyte apical membrane by the divalent metal transporter (DMT1). In the enterocyte, Fe2+is then either stored in enterocyte epithelial cells as ferritin, or released into portal plasma via the molecule ferroportin at the basolateral membrane.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 7 - How does aspirin mediate its antiplatelet effect: ...

    Incorrect

    • How does aspirin mediate its antiplatelet effect:

      Your Answer:

      Correct Answer: It irreversibly inhibits cyclo-oxygenase

      Explanation:

      Aspirin irreversibly inhibits cyclooxygenase and blocks the platelet production of thromboxane A2 (TXA2), thus inhibiting platelet aggregation.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 8 - Regarding acute idiopathic thrombocytopaenic purpura (ITP), which of the following statements is CORRECT:...

    Incorrect

    • Regarding acute idiopathic thrombocytopaenic purpura (ITP), which of the following statements is CORRECT:

      Your Answer:

      Correct Answer: Over 80% of children recover without treatment.

      Explanation:

      Acute ITP is most common in children. In approximately 75% of cases, the episode follows vaccination or infection such as chicken pox or glandular fever. Most cases are caused by non-specific immune complex attachment to platelets. Acute ITP usually has a very sudden onset and the symptoms usually disappear in less than 6 months (often within a few weeks). It is usually a self-limiting condition and over 80% of children recover without treatment; in 5 – 10% of cases a chronic form of the disease develops.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 9 - Which of the following leukaemias is most common in children in the UK:...

    Incorrect

    • Which of the following leukaemias is most common in children in the UK:

      Your Answer:

      Correct Answer: Acute lymphoblastic leukaemia

      Explanation:

      Acute lymphoblastic leukaemia (ALL) is caused by an accumulation of lymphoblasts in the bone marrow and is the most common malignancy of childhood. The incidence of ALL is highest at 3 – 7 years, with 75% of cases occurring before the age of 6. There is a secondary rise after the age of 40 years. 85% of cases are of B-cell lineage and have an equal sex incidence; there is a male predominance for the 15% of T-cell lineage.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 10 - Aplastic crises in sickle cell anaemia is classically precipitated by which of the...

    Incorrect

    • Aplastic crises in sickle cell anaemia is classically precipitated by which of the following infections:

      Your Answer:

      Correct Answer: Parvovirus B19

      Explanation:

      A serious complication in sickle cell disease (SCD) is the aplastic crisis. This may be caused by infection with Parvovirus B-19 (B19V). This virus causes fifth disease, a normally benign childhood disorder associated with fever, malaise, and a mild rash. This virus infects RBC progenitors in bone marrow, resulting in impaired cell division for a few days.
      Healthy people experience, at most, a slight drop in hematocrit, since the half-life of normal erythrocytes in the circulation is 40-60 days. In people with SCD, however, the RBC lifespan is greatly shortened (usually 10-20 days), and a very rapid drop in Hb occurs. The condition is self-limited, with bone marrow recovery occurring in 7-10 days, followed by brisk reticulocytosis.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 11 - Regarding iron deficiency anaemia, which of the following statements is INCORRECT: ...

    Incorrect

    • Regarding iron deficiency anaemia, which of the following statements is INCORRECT:

      Your Answer:

      Correct Answer: Dietary insufficiency is the most common cause of iron deficiency anaemia in adult men in the UK.

      Explanation:

      Blood loss from the gastrointestinal (GI) tract is the most common cause of iron deficiency anaemia in adult men and postmenopausal women.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 12 - A medical student is reviewing the chart of a patient who has recently...

    Incorrect

    • A medical student is reviewing the chart of a patient who has recently been diagnosed with leukaemia. The chief resident asks him to identify ONE feature that will most support acute leukaemia diagnosis in contrast to chronic leukaemia.

      Which one of the following options will he pick?

      Your Answer:

      Correct Answer: The patient is a child

      Explanation:

      Acute lymphocytic leukaemia (ALL) is more common in children than chronic leukaemias, which generally occur in adults. Bone marrow failure occurs early on in the course of the disease in acute leukaemias, and there is the massive proliferation of undifferentiated cells with functioning cells being crowded out. Hepatosplenomegaly occurs in both acute and chronic forms of leukaemia and is not a differentiating feature.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 13 - A 49-year-old man known sickle cell disease patient received a blood transfusion and...

    Incorrect

    • A 49-year-old man known sickle cell disease patient received a blood transfusion and developed a mild fever and dark urine 6 days after transfusion. His investigations show raised bilirubin, raised LDH, and a positive Direct Antiglobulin Test (DAT).

      Which transfusion reaction is most likely occurred?

      Your Answer:

      Correct Answer: Delayed haemolytic reaction

      Explanation:

      Delayed haemolytic transfusion reactions (DHTRs) commonly occurs 4-8 days after blood transfusion, but can occur up to a month after. Signs and symptoms include jaundice, fever, an inadequate rise in PCV, reticulocytosis, a positive antibody screen and a positive Direct Antiglobulin Test (Coombs test). DHTRs usually have a benign course and require no treatment but sometimes, life-threatening haemolysis with severe anaemia and renal failure can occur so haemoglobin levels and renal function should be monitored.

      Acute haemolytic reaction aka immediate haemolytic transfusion reaction presents with fever, chills, pain at transfusion site, nausea, vomiting, dark urine and feeling of ‘impending doom’. Often, it occurs due to ABO incompatibility.

      Febrile transfusion reaction presents with a 1 degree rise in temperature from baseline during transfusion. Patient may have chills and malaise. It is the most common transfusion reaction (1 in 8 transfusions) and is usually caused by cytokines released from leukocytes in transfused red cell or platelet components.

      Transfusion-associated circulatory overload (TACO) presents as acute or worsening respiratory distress within 6 hours of transfusion of a large volume of blood. It is common in patients with diminished cardiac reserve or chronic anaemia. Elderly patients, infants and severely anaemic patients are particularly susceptible. Clinical features of TACO include: Acute respiratory distress, Tachycardia, Hypertension, Acute/worsening pulmonary oedema on chest X-ray. The BNP is usually raised to at least 1.5 times the pre-transfusion baseline.

      Graft versus host disease(GVHD) is an immune mediated condition that arises from a complex interaction between donor and recipients adaptive immunity. It presents as dermatitis, hepatitis and enteritis developing within 100 days after stem cell or bone marrow transplant.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 14 - Which of the following statements about sickle cell disease is TRUE: ...

    Incorrect

    • Which of the following statements about sickle cell disease is TRUE:

      Your Answer:

      Correct Answer: Hand-foot syndrome is frequently a first presentation of the disease.

      Explanation:

      Hand-foot syndrome in children is typically the first symptom of the disease, produced by infarction of the metaphysis of small bones. The disease is inherited as an autosomal recessive trait. By adulthood, the spleen has usually infarcted. Infection with the B19 parvovirus is usually followed by an aplastic crisis. Thrombocytopenia is caused by splenic sequestration.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 15 - Regarding Hodgkin lymphoma, which of the following statements is CORRECT: ...

    Incorrect

    • Regarding Hodgkin lymphoma, which of the following statements is CORRECT:

      Your Answer:

      Correct Answer: Some patients may complain alcohol-induced nodal pain and pruritus

      Explanation:

      Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and other lymphoid tissue and cause the characteristic clinical feature of lymphadenopathy. The major subdivision of lymphomas is into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) and this is based on the histological presence of Reed-Sternberg cells present in HL. Hodgkin lymphoma can present at any age but is rare in children and has a peak incidence in young adults. There is an almost 2 : 1 male predominance. Most patients present with painless, asymmetrical, firm and discrete enlargement of superficial lymph nodes. Cervical nodes are involved in 60-70% of cases, axillary nodes in 10-15% and inguinal nodes in 6-12%. Modest splenomegaly occurs during the course of the disease in 50% of patients; the liver may also be enlarged. Bone marrow failure involvement is unusual in early disease. The prognosis depends on age, stage and histology, but overall approximately 85% of patients are cured. Alcohol‐induced pain and pruritus are two well‐known but rare symptoms in HL.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 16 - A 60-year-old female is referred to the Oncology clinic due to the presence...

    Incorrect

    • A 60-year-old female is referred to the Oncology clinic due to the presence of lumps in her neck. There is a non-tender enlargement of several groups of cervical lymph nodes on examination. She is sent for a lymph node biopsy. The results show the presence of lymphoma cells, but there are no Reed-Sternberg cells.

      Which one is most appropriate for this case out of the following diagnoses?

      Your Answer:

      Correct Answer: Non-Hodgkin’s lymphoma

      Explanation:

      Non-Hodgkin’s Lymphoma (NHL) causes neoplastic transformation of both B cell (85%) and T cell (15%) lines.

      The most common presentation is with enlarged, rubbery, painless lymph nodes. The patient may also have B symptoms which consist of night sweats, weight loss and fevers. Multiple myeloma most commonly presents with bone pain, especially in the back and ribs.

      The presence of Reed-Sternberg cells characterises Hodgkin’s lymphoma. Acute lymphoblastic leukaemia will present with features of anaemia, thrombocytopenia and leukopenia. The most common symptoms of chronic lymphocytic leukaemia are fatigue, night sweats and low-grade fever.

      The peak incidence of NHL is in the 50-70 years age group, it affects men and women equally, but affects the Caucasian population more commonly than black and Asian ethnic groups.
      The following are recognised risk factors for NHL:
      Chromosomal translocations and molecular rearrangements
      Epstein-Barr virus infection
      Human T-cell leukaemia virus type-1 (HTLV-1)
      Hepatitis C
      Congenital and acquired immunodeficiency states
      Autoimmune disorders, e.g. Sjogren’s syndrome and Hashimoto’s thyroiditis

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 17 - A 61-year-old woman returns to get the results of recent blood tests she...

    Incorrect

    • A 61-year-old woman returns to get the results of recent blood tests she had done for non-specific malaise, lethargy, and weight loss.

      The only abnormality discovered during the blood tests was a 580 x 10 9 /l increase in platelets. Her platelets were also elevated on a blood test taken 6 months earlier, according to her previous results. You're aware of the recent emergence of elevated platelet levels as a cancer risk marker and decide to look into it.

      Which of the following cancers is most likely to cause isolated thrombocytosis?

      Your Answer:

      Correct Answer: Colorectal cancer

      Explanation:

      Raised platelet levels have emerged as a cancer risk marker, according to a large population-based study published in 2017(link is external). According to the study, 12 percent of men and 6% of women with thrombocytosis were diagnosed with cancer within a year. These figures increased to 18% in men and 10% in women if a second platelet count was taken within 6 months of the first and showed an increased or stable elevated platelet count.

      The researchers discovered that thrombocytosis linked to cancer is most common in colorectal and lung cancers, and it is linked to a worse prognosis. Furthermore, one-third of the cancer patients in the study had no other symptoms that would have prompted an immediate cancer referral.

      The exact mechanism by which these cancers cause thrombocytosis is unknown, but one theory proposes the existence of pathogenic feedback loops between malignant cells and platelets, with a reciprocal interaction between tumour growth and metastasis, as well as thrombocytosis and platelet activation. Another hypothesis is that thrombocytosis occurs independently of cancer but aids in its spread and progression.

      The findings show that routinely testing for thrombocytosis could cut the time it takes to diagnose colorectal and lung cancer by at least two months. In the UK, this could result in around 5500 earlier cancer diagnoses per year.

      Because the positive predictive value of thrombocytosis in middle age for cancer (10%) is higher than the positive predictive value for a woman in her 50s presenting with a new breast lump (8.5%), this is clearly an important research paper that should be used to adjust future clinical practise. The current NICE guidelines predate these new research findings, so we’ll have to wait and see how they affect cancer referral guidelines in the UK.

      Because there are so many possible cancers associated with thrombocytosis, the treating clinician should take a thorough history and perform a thorough clinical examination if a patient is diagnosed with it. Further investigation and the most appropriate referral route should be aided by this information.

      It’s worth noting that the patients in the study had their blood tests done for a medical reason rather than as a random screening test.

      If there are no other symptoms to guide investigation and referral (one-third of the patients in the study had no other symptoms), keep in mind that the two most common cancers encountered were colorectal and lung cancer, so a chest X-ray and a faecal immunochemical test (FIT) for faecal blood may be reasonable initial investigations.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 18 - Regarding acute lymphoblastic leukaemia (ALL), which of the following statements is CORRECT: ...

    Incorrect

    • Regarding acute lymphoblastic leukaemia (ALL), which of the following statements is CORRECT:

      Your Answer:

      Correct Answer: 75% of cases occur before the age of 6 years.

      Explanation:

      Acute lymphoblastic leukaemia (ALL) is caused by an accumulation of lymphoblasts in the bone marrow and is the most common malignancy of childhood. The incidence of ALL is highest at 3 – 7 years, with 75% of cases occurring before the age of 6. 85% of cases are of B-cell lineage. Haematological investigations reveal a normochromic normocytic anaemia with thrombocytopenia in most cases. There is great variation in the chance of individual patients achieving a long-term cure based on a number of biological variables. Approximately 25% of children relapse after first-line therapy and need further treatment but overall 90% of children can expect to be cured. The cure rate in adults drops significantly to less than 5% over the age of 70 years.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
      Seconds
  • Question 19 - A 66-year-old male presents to his family physician with the complaint of increasing...

    Incorrect

    • A 66-year-old male presents to his family physician with the complaint of increasing fatigue and lethargy, along with itching, especially after a hot bath. He also complains of increased sweating and dizziness. On examination, he has a plethoric appearance. Abdominal examination shows the presence of splenomegaly. A basic panel of blood tests is ordered in which her Hb comes out to be 17 g/dL.

      Which one of the following treatment options will be most suitable in this case?

      Your Answer:

      Correct Answer: Venesection

      Explanation:

      The clinical and laboratory findings, in this case, support a diagnosis of polycythaemia vera. A plethoric appearance, lethargy, splenomegaly and itching are common in this disease. Patients may also have gouty arthritis, Budd-Chiari syndrome, erythromelalgia, stroke, myocardial infarction or DVT. The average age for diagnosis of Polycythaemia Vera is 65-74 years. It is a haematological malignancy in which there is overproduction of all three cell lines. Venesection is the treatment of choice as it would cause a decrease in the number of red blood cells within the body.

      Erythropoietin is given in patients with chronic renal failure as they lack this hormone. Administration of erythropoietin in such patients causes stimulation of the bone marrow to produce red blood cells.

      Desferrioxamine is a chelating agent for iron and is given to patients with iron overload due to repeated blood transfusions, e.g. in thalassemia patients.

      Penicillamine is a chelating agent for Copper, given as treatment in Wilson’s disease.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
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  • Question 20 - A 49-year-old woman with haemoglobin of 6 g/dL following persistent vaginal bleeding receives...

    Incorrect

    • A 49-year-old woman with haemoglobin of 6 g/dL following persistent vaginal bleeding receives blood transfusion. She developed pain and burning at her cannula site and complains of a feeling of “impending doom”, nausea, and severe back pain shortly after transfusion was started. Her temperature is 38.9ºC.

      What is the most appropriate treatment?

      Your Answer:

      Correct Answer: Stop the transfusion and administer IV fluids

      Explanation:

      Acute haemolytic transfusion reactions present with: Feeling of ‘impending doom’ as the earliest symptom, fever and chills, pain and warmth at transfusion site, nausea and vomiting, back, joint, and chest pain. Transfusion should be stopped immediately and IV fluid (usually normal saline) administered.

      Supportive measures and paracetamol can be given since patient has fever but it is not the immediate first step.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 21 - A 35-year-old man suffered a severe road traffic accident and has been brought...

    Incorrect

    • A 35-year-old man suffered a severe road traffic accident and has been brought to the hospital. As part of his treatment, he requires a blood transfusion and experiences a transfusion reaction.

      The most common type of transfusion reaction is which of the following?

      Your Answer:

      Correct Answer: Febrile transfusion reaction

      Explanation:

      During or shortly after transfusion, febrile transfusion reactions, also known as non-haemolytic transfusion reactions, present with an unexpected temperature rise (38oC or 1oC above baseline, if baseline is 37oC). This is usually a one-off occurrence. The fever is sometimes accompanied by chills.

      The most common type of transfusion reaction is febrile transfusion reactions, which occur in about 1 in every 8 transfusions.

      The most common event leading to symptoms of febrile transfusion reactions is cytokine accumulation during storage of cellular components (especially platelet units). White cells secrete cytokines, and pre-storage leucodepletion has reduced this risk.

      Recipient antibodies (raised as a result of previous transfusions or pregnancies) reacting to donor human leukocyte antigen (HLA) or other antigens can also cause febrile transfusion reactions. Donor lymphocytes, granulocytes, and platelets all contain these antigens.

      Treatment is reassuring. Other causes should be ruled out, and antipyretics like paracetamol can help with fever relief. If another cause of fever is suspected, the transfusion should be stopped; however, if other causes of fever have been ruled out, it can be restarted at a slower rate.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 22 - Which of the following is NOT a typical clinical feature of sickle cell...

    Incorrect

    • Which of the following is NOT a typical clinical feature of sickle cell disease:

      Your Answer:

      Correct Answer: Neutropaenia

      Explanation:

      Features of sickle cell disease include:
      Anaemia (symptoms are usually mild because the O2 dissociation curve of Hb S is shifted to the right)
      Vaso-occlusive crisis
      Visceral sequestration crisis
      Aplastic crisisIncreased susceptibility to infection
      Other clinical features: Pigment gallstones with cholecystitis
      Chronic leg ulcers
      Avascular necrosis of the femoral and humeral heads or other bones
      Cardiomyopathy
      Pulmonary hypertension
      Proliferative retinopathy
      Priapism
      Renal papillary necrosis
      Stroke

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 23 - A 16-year-old male with a known case of Haemophilia A is referred to...

    Incorrect

    • A 16-year-old male with a known case of Haemophilia A is referred to your orthopaedic clinic for evaluation and aspiration of a hemarthrosis of the left knee joint.

      Out of the modes of inheritance listed below, which one is present in this disease?

      Your Answer:

      Correct Answer: X-linked recessive

      Explanation:

      All the Haemophilia’s have an X-linked recessive inheritance pattern, so they only manifest in male patients. Diseases with a mitochondrial inheritance pattern include MELAS syndrome, Leigh syndrome, LHON and MERRF syndrome. Autosomal dominant disorders include Huntingdon disease and Marfan syndrome. X-linked dominant diseases include Fragile X syndrome. Autosomal recessive diseases include cystic fibrosis and sickle cell disease.

    • This question is part of the following fields:

      • Haematology
      • Pathology
      0
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  • Question 24 - Which of the following clotting factors is NOT vitamin K-dependent: ...

    Incorrect

    • Which of the following clotting factors is NOT vitamin K-dependent:

      Your Answer:

      Correct Answer: V

      Explanation:

      Fat-soluble vitamin K is obtained from green vegetables and bacterial synthesis in the gut. Deficiency may present in the newborn (haemorrhagic disease of the newborn) or in later life. Deficiency may be caused by an inadequate diet, malabsorption or inhibition of vitamin K by drugs such as warfarin. The activity of factors II, VII, IX and X are vitamin K dependent as well as that of protein C and protein S. Both PT and APTT are prolonged.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 25 - A 66-year-old male is brought to your clinic by his wife with the...

    Incorrect

    • A 66-year-old male is brought to your clinic by his wife with the complaint of increasing confusion and disorientation over the past three days, along with decreased urination. She reports that he has been complaining of increasing pain in his back and ribs over the past three months.

      On examination, the patient looks anaemic, is not oriented in time, place and person and has tenderness on palpation of the lumbar spine and the 10th, 11th and 12th ribs.

      Blood tests show anaemia which is normocytic, normochromic, raised urea and creatinine and hypercalcemia.

      Which one of the following diagnoses is most likely in this case?

      Your Answer:

      Correct Answer: Multiple myeloma

      Explanation:

      Multiple myeloma is a clonal abnormality affecting plasma cells in which there is an overproduction of functionless immunoglobulins. The most common patient complaint is bone pain, especially in the back and ribs. Anaemia and renal failure are common, along with hypercalcemia. Hypercalcemia may lead to an altered mental status, as in this case.

      Chronic lymphocytic leukaemia occurs due to the overproduction of lymphocytes, usually B cells. CLL may present with an asymptomatic elevation of B cells. Patients are generally more than 50 years old and present with non-specific fatigue and weight loss symptoms.

      There is no history of alcohol abuse in this case. Furthermore, patients with a history of alcohol abuse may have signs of liver failure, which are not present here.

      Metastatic prostate cancer would most often cause lower backache as it metastasises first to the lumbar spine via the vertebral venous plexus.

      A patient with Vitamin B12 deficiency would have anaemia, megaloblastic, hypersegmented neutrophils, and signs of peripheral neuropathy.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 26 - A 30-year-old woman is transfused following a diagnosis of anaemia secondary to heavy...

    Incorrect

    • A 30-year-old woman is transfused following a diagnosis of anaemia secondary to heavy vaginal bleeding. She complains of feeling hot and cold during transfusion of the second unit and her temperature is 38.5ºC. Prior to the transfusion, her temperature was 37ºC. She has no other symptoms.

      Which of these transfusions reactions most likely occurred?

      Your Answer:

      Correct Answer: Febrile transfusion reaction

      Explanation:

      Febrile transfusion reactions presents with an unexpected temperature rise (≥ 38ºC or ≥ 1ºC above baseline, if baseline ≥ 37ºC) during or shortly after transfusion. It is usually an isolated finding and the fever is accompanied by chills and malaise occasionally.

      Allergic reaction is commonly caused by foreign plasma proteins but may be due to anti-IgA. Allergic type reactions usually present with urticaria, pruritus, hives. Associations include laryngeal oedema or bronchospasm.

      Acute haemolytic reaction aka immediate haemolytic transfusion reaction presents with fever, chills, pain at transfusion site, nausea, vomiting, dark urine and feeling of ‘impending doom’. Often, it occurs due to ABO incompatibility.

      Transfusion-associated circulatory overload (TACO) presents as acute or worsening respiratory distress within 6 hours of transfusion of a large volume of blood. It is common in patients with diminished cardiac reserve or chronic anaemia. Elderly patients, infants and severely anaemic patients are particularly susceptible. Clinical features of TACO include: Acute respiratory distress, Tachycardia, Hypertension, Acute/worsening pulmonary oedema on chest X-ray. The BNP is usually raised to at least 1.5 times the pre-transfusion baseline.

      Transfusion-related lung injury (TRALI) is a form of acute respiratory distress caused by the donor plasma containing antibodies against the patient’s leukocytes. It is defined as hypoxia and bilateral pulmonary oedema that occurs within 6 hours of a transfusion in the absence of other causes of acute lung injury. Clinical features include Breathlessness, cough, frothy sputum, hypertension or hypotension, hypoxia and fever. Chest X-ray shows multiple perihilar nodules with infiltration of the lower lung fields.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 27 - You examine a 78-year-old man who has been diagnosed with chronic lymphocytic leukaemia...

    Incorrect

    • You examine a 78-year-old man who has been diagnosed with chronic lymphocytic leukaemia (CLL).

      What is the MAIN contributory factor in this condition's immunodeficiency?

      Your Answer:

      Correct Answer: Hypogammaglobulinemia

      Explanation:

      Immunodeficiency is present in all patients with chronic lymphocytic leukaemia (CLL), though it is often mild and not clinically significant. Infections are the leading cause of death in 25-50 percent of CLL patients, with respiratory tract, skin, and urinary tract infections being the most common.

      Hypogammaglobulinemia is the most common cause of immunodeficiency in CLL patients, accounting for about 85 percent of all cases.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 28 - The least likely feature of anaemia is: ...

    Incorrect

    • The least likely feature of anaemia is:

      Your Answer:

      Correct Answer: Narrow pulse pressure

      Explanation:

      Non-specific signs of anaemia include:
      1. pallor of mucous membranes or nail beds (if Hb < 90 g/L),
      2. tachycardia
      3. bounding pulse
      4. wide pulse pressure
      5. flow murmurs
      6. cardiomegaly
      7. signs of congestive cardiac failure (in severe cases)

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 29 - A 26-year-old male presents to your clinic with the complaint of recurrent fevers...

    Incorrect

    • A 26-year-old male presents to your clinic with the complaint of recurrent fevers over the past three months. On documentation of the fever, it is noted that the fever increases and decreases in a cyclical pattern over a 1-2 week period. After the appropriate investigations, a diagnosis of Pel-Ebstein fever is made.

      Which ONE of the following conditions is most likely to cause this patient's fever?

      Your Answer:

      Correct Answer: Hodgkin lymphoma

      Explanation:

      The release of cytokines from Reed-Sternberg cells can cause fever in patients with Hodgkin lymphoma, which increases and decreases in a cyclical pattern of 1 to 2 weeks. This is called Pel-Ebstein or Ebstein-Cardarelli fever, specifically seen in Hodgkin lymphoma. The fever is always high grade and can reach 40 degrees or higher.

      Cyclical fever in other conditions is common but is not termed as Pel-Ebstein fever. This term is reserved only with Hodgkin lymphoma.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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  • Question 30 - A 1-year-old male is brought to the paediatrician by his mother due to...

    Incorrect

    • A 1-year-old male is brought to the paediatrician by his mother due to swelling of the right knee after a minor fall. On examination, the right knee is swollen, fluctuant and tender. Ultrasound-guided aspiration reveals a massive hemarthrosis. Family history shows that his older brother also has a bleeding disorder.

      Which one of the following conditions does the patient most likely have?

      Your Answer:

      Correct Answer: Haemophilia A

      Explanation:

      A diagnosis of Haemophilia is supported in this patient by the family history and the presence of hemarthrosis-both characteristics of Haemophilia. Haemophilia A is caused by Factor VIII deficiency, leading to impaired coagulation. This disease typically presents after six months when the child starts crawling.

      Von Willebrand disease presents with nosebleeds and hematomas. Idiopathic thrombocytopenic purpura presents with bruises that resemble a rash.

      Glucose-6-phosphate dehydrogenase (G6PD) deficiency presents with haemolytic anaemia induced by specific drugs or foods.

      Factor V Leiden mutation causes blood clotting rather than bleeding.

    • This question is part of the following fields:

      • Haematology
      • Pathology
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