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Question 1
Correct
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A 35-year old lady presents to her GP with vague abdominal symptoms. Examination reveals a normal size spleen. Which of the following is the likely diagnosis?
Your Answer: Idiopathic thrombocytopenic purpura
Explanation:Idiopathic thrombocytopenic purpura (ITP) is a disease caused due to development of an antibody against a platelet antigen (autoantibody). In childhood disease, the autoantibody gets triggered by binding of viral antigen to the megakaryocytes. Presentation includes unexplained thrombocytopenia, petechiae and bleeding from mucosal surfaces. The spleen usually does not enlarge in size. However, splenomegaly can occur due to coexisting viral infection. Marrow examination reveals normal or increased number of megakaryocytes. Diagnosis is by exclusion of other thrombocytopenic disorders.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 2
Correct
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Investigations in a 40-year old gentleman with splenomegaly reveal the following: haemoglobin 21.5 g/dl, haematocrit 66%, mean corpuscular volume (MCV) 86 fl, mean cell haemoglobin concentration 34 g/dl, mean corpuscular haemoglobin 34.5 pg, platelet count 450 × 109/l, and white blood cell count 12 × 109/l, with 81% polymorphonuclear leukocytes, 4% bands, 3% monocytes, and 7% lymphocytes.
What is the likely diagnosis?Your Answer: Polycythaemia vera
Explanation:The markedly increased haematocrit, along with thrombocytosis and the leucocytosis suggest a myeloproliferative disorder.
Polycythaemia vera is the commonest myeloproliferative disorders occurring more often in males (about 1.4 to 1). The mean age at diagnosis is 60 years (range 15–90 years) with 5% of patients below 40 years at onset. It involves increased production of all cell lines, including red blood cells (independent of erythropoietin), white blood cells and platelets. If confined only to red blood cells, it is known as ‘primary erythrocytosis’. There is an increase in blood volume and hyperviscosity occurs, predisposing to thrombosis. Increased bleeding occurs due to abnormal functioning of platelets. Patients become hypermetabolic, and increased cell turnover leads to hyperuricaemia.
Usually asymptomatic, occasionally symptoms include weakness, pruritus, headache, light-headedness, visual disturbances, fatigue and dyspnoea. Face appears red with engorged retinal veins. Lower extremities appear red and painful, along with digital ischaemia (erythromelalgia). Hepatomegaly is common and massive splenomegaly is seen in 75% patients. Thrombosis can lead to stroke, deep venous thrombosis, myocardial infarction, retinal artery or vein occlusion, splenic infarction (often with a friction rub) or Budd–Chiari syndrome. Gastrointestinal bleeding is seen in 10-20% patients. Hypermetabolism can lead to low-grade fevers and weight loss. Late features include complications of hyperuricaemia (e.g. gout, renal calculi). 1.5% to 10% cases transform to acute leukaemia.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 3
Incorrect
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A 24-year-old patient with recurrent episodes of deep vein thrombosis presents again to the clinic. Deficiency of which of the following blood proteins is the most probable cause of this episode?
Your Answer: Fibrinogen
Correct Answer: Antithrombin III
Explanation:Antithrombin III (ATIII) is a blood protein that acts by inhibiting blood coagulation by neutralizing the enzymatic activity of thrombin.
Antithrombin III deficiency is an autosomal dominant disorder that leads to an increased risk of venous and arterial thrombosis. Clinical manifestations typically appear in young adulthood.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 4
Correct
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An enlarged lymph node which shows well-defined, prominent paracortical follicles with germinal centres is most likely from which of the following patients?
Your Answer: A 5-year-old boy with a sore throat and runny nose
Explanation:Lymphadenopathy is common in children and is usually reactive in nature. The description fits that of a benign, reactive lymph node.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 5
Correct
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Which of the following over-the-counter drugs can cause a prolonged bleeding time?
Your Answer: Acetylsalicylic acid
Explanation:Acetylsalicylic acid, or aspirin, is a nonsteroidal anti-inflammatory drug that is widely used as an analgesic and antipyretic. Aspirin is as a cyclo-oxygenase inhibitor that leads to decreased prostaglandin production. Decreased platelet aggregation is another effect of this drug, achieved by long-lasting use of aspirin.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 6
Correct
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Question 7
Incorrect
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A 40-year old lady presented to the hospital with fever and mental confusion for 1 week. On examination, she was found to have multiple petechiae all over her skin and mucosal surfaces. Blood investigations revealed low platelet count and raised urea and creatinine. A platelet transfusion was carried out, following which she succumbed to death. Autopsy revealed pink hyaline thrombi in myocardial arteries. What is the likely diagnosis?
Your Answer: Idiopathic thrombocytopenic purpura
Correct Answer: Thrombotic thrombocytopenic purpura
Explanation:Hyaline thrombi are typically associated with thrombotic thrombocytopenic purpura (TTP), which is caused by non-immunological destruction of platelets. Platelet transfusion is contraindicated in TTP. Platelets and red blood cells also get damaged by loose strands of fibrin deposited in small vessels. Multiple organs start developing platelet-fibrin thrombi (bland thrombi with no vasculitis) typically at arteriocapillary junctions. This is known as ‘thrombotic microangiopathy’. Treatment consists of plasma exchange.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 8
Correct
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Elevated mean corpuscular volume with hypersegmented neutrophils and low reticulocyte index is seen in on the blood count of a middle-aged lady about to undergo elective surgery. On enquiry, she mentions feeling tired for a few months. Which of the following investigations should be carried out in her to reach a diagnosis?
Your Answer: Serum vitamin B12 and folate
Explanation:Elevated levels of MCV indicates megaloblastic anaemia, which are associated with hypersegmented neutrophils. Likely causes include vitamin B12 or folate deficiency. Megaloblastic anaemia results from defective synthesis of DNA. As RNA production continues, the cells enlarge with a large nucleus. The cytoplasmic maturity becomes greater than nuclear maturity. Megaloblasts are produced initially in the marrow, before blood. Dyspoiesis makes erythropoiesis ineffective, causing direct hyperbilirubinemia and hyperuricemia. As all cell lines are affected, reticulocytopenia, thrombocytopenia and leukopenia develop. Large, oval blood cells (macro-ovalocytes) are released in the circulation, along with presence of hypersegmented neutrophils.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 9
Correct
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Skin infiltration by neoplastic T lymphocytes is seen in:
Your Answer: Mycosis fungoides
Explanation:Mycosis fungoides is a chronic T-cell lymphoma that involves the skin and less commonly, the internal organs such as nodes, liver, spleen and lungs. It is usually diagnosed in patients above 50 years and the average life expectancy is 7-10 years. It is insidious in onset and presents as a chronic, itchy rash, eventually spreading to involve most of the skin. Lesions are commonly plaque-like, but can be nodular or ulcerated. Symptoms include fever, night sweats and weight loss. Skin biopsy is diagnostic. However, early cases may pose a challenge due to fewer lymphoma cells. The malignant cells are mature T cells (T4+, T11+, T12+). The epidermis shows presence of characteristic Pautrier’s micro abscesses are present in the epidermis.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 10
Correct
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The absence of which of the following components characterizes the grey platelet syndrome (GPS)?
Your Answer: Alpha granules
Explanation:Grey platelet syndrome (GPS) is a rare inherited bleeding disorder associated with an almost total absence of α-granules and their contents. The syndrome is characterised by thrombocytopenia, enlarged platelets that have a grey appearance, myelofibrosis, and splenomegaly. Alpha granules store proteins and growth factors that promote platelet adhesiveness and wound healing. Patients with GPS develop symptoms and signs such as easy bruising, prolonged bleeding, and nose bleeds.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 11
Incorrect
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Which of the following proteins acts as cofactor in the thrombin-induced activation of anticoagulant protein C?
Your Answer: Fibronectin
Correct Answer: Thrombomodulin
Explanation:Thrombomodulin is a protein cofactor expressed on the surface of endothelial cells. Thrombomodulin binds with thrombin forming a complex which activates protein C, initiating the anticoagulant pathway.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 12
Correct
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A 14 year-old girl is found to have haemophilia B. What pathological problem does she have?
Your Answer: Deficiency of factor IX
Explanation:Haemophilia B (also known as Christmas disease) is due to a deficiency in factor IX. Haemophilia A is due to a deficiency in factor VIII.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 13
Incorrect
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From which of the following cells is heparin produced?
Your Answer: Endothelial cells
Correct Answer: Mast cells
Explanation:Heparin is a natural highly-sulphated glycosaminoglycan that has anticoagulant functions. It is produced by the body basophils and mast cells.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 14
Incorrect
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Coagulation in the body (in vivo) is a process in which several proteins known as coagulation factors are activated in a cascade effect to stop bleeding. Which of the following initiates this cascade effect?
Your Answer: Factor VII
Correct Answer: Tissue factor
Explanation:Tissue factor (TF), also known as ‘factor III’ or ‘thromboplastin’, is an anti-coagulation protein that initiates the extrinsic coagulation. TF acts as a transmembrane receptor for Factor VII/VIIa . It is expressed by endothelial cells but also certain tissues, such as the heart and brain.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 15
Incorrect
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A 50-year old gentleman who was admitted for elective surgery was found to have haemoglobin 9.5 g/dl, white blood cell count of 1.4 × 109/l and a mean corpuscular volume (MCV) of 134 fl. Which of the following is the likely finding on his peripheral blood smear?
Your Answer: Schistocytes
Correct Answer: Hypersegmented neutrophils
Explanation:The likely diagnosis is megaloblastic anaemia, which also shows the presence of hypersegmented neutrophils.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 16
Incorrect
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A 17-year-old boy, who had developed shortness of breath and a loss of appetite over the last month, was referred to a haematologist because he presented with easy bruising and petechiae. His prothrombin time, platelet count, partial thromboplastin and bleeding time were all normal. Which of the following would explain the presence of the petechiae and easy bruising tendency?
Your Answer: Uraemia
Correct Answer: Scurvy
Explanation:Scurvy is a condition caused by a dietary deficiency of vitamin C, also known as ascorbic acid. Humans are unable to synthesize vitamin C, therefore the quantity of it that the body needs has to come from the diet. The presence of an adequate quantity of vitamin C is required for normal collagen synthesis. In scurvy bleeding tendency is due to capillary fragility and not coagulation defects, therefore blood tests are normal.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 17
Correct
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A 20-year old boy presented with low grade fever, night sweats and weakness over two months. On examination, he had multiple, non-tender, cervical, supraclavicular and axillary adenopathy. Microscopy of lymph node biopsy showed the presence of Reed-Sternberg cells. He is likely suffering from:
Your Answer: Hodgkin’s lymphoma
Explanation:Hodgkin’s lymphoma is a disease characterized by malignant proliferation of cells of the lymphoreticular system. It can be localized or disseminated, and can involve the nodes, spleen, liver and marrow. Symptoms of the disease include non-tender lymphadenopathy, fever, night sweats, weight loss, itching and hepatosplenomegaly. Histologically, the involved nodes show the presence of Reed-Sternberg cells, which are large, binucleated cells, in a heterogenous cellular infiltrate of histiocytes, lymphocytes, monocytes, plasma cells and eosinophils.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 18
Incorrect
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Low molecular weight heparin (LMWH) has less side effects than heparin and is used in the prophylaxis and treatment of venous and arterial thrombotic disorders. Which of the following is LMWHs mechanism of action?
Your Answer: Inhibition of antithrombin III
Correct Answer: Inhibition of factor Xa
Explanation:Low molecular weight heparin (LMWH) is a anticoagulant that differs from normal heparin in that it has only short chains of polysaccharide. LMWH inhibits thrombin formation by converting antithrombin from a slow to a rapid inactivator of coagulation factor Xa.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 19
Correct
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A 36-year old gentleman with a history of cough for 4 weeks came to the hospital. Examination revealed multiple lymphadenopathy with splenomegaly. Investigations revealed haemoglobin 11 g/dl, haematocrit 32.4%, mean corpuscular volume (MCV) 93 fl, white blood cell count 63 × 109/l, and platelet count 39 × 109/l; along with characteristic Auer rods on peripheral blood smear. What is the likely diagnosis?
Your Answer: Acute myelogenous leukaemia (AML)
Explanation:AML, or acute myeloid leukaemia is the commonest acute leukaemia affecting adults. increasing in incidence with age. It is a malignancy of the myeloid line of white blood cells. It results in rapid proliferation of abnormal cells, which accumulate in the marrow. Interference with normal cell production leads to a drop in red blood cells, white blood cells and platelets. This causes symptoms such as tiredness, shortness of breath, tendency to bleed or bruise easily and recurrent infections. AML is known to progress quickly and can lead to death in weeks and months if not treated. Leukemic blasts of AML show presence of Auer rods. These are clumps of azurophilic granular material that form needles in the cytoplasm. Composed of fused lysosomes, these contain peroxidase, lysosomal enzymes and crystalline inclusions. Auer rods are classically present in myeloid blasts of M1, M2, M3 and M4 acute leukaemia. They also help to distinguish the preleukemia myelodysplastic syndromes.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 20
Correct
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Question 21
Correct
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Which of the following conditions is likely to result in splenomegaly, hypochromic anaemia and hemochromatosis in a young male?
Your Answer: β-Thalassaemia
Explanation:Beta-thalassaemia is due to decreased production of β-polypeptide chains, with an autosomal inheritance pattern. Carrier patients (heterozygotes) are asymptomatic and have mild to moderate microcytic anaemia. This is known as thalassaemia minor. Homozygotes (β-thalassaemia major, or Cooley’s anaemia) develop severe anaemia and marrow hyperactivity. The disease presents at 1-2 years of age with severe anaemia and transfusional and absorptive iron overload. Patients also present with jaundice, leg ulcers, massive splenomegaly and cholelithiasis. The disease can also lead to splenic sequestration leading to faster destruction of transfused red blood cells. Increased marrow activity causes thickening of cranial bones. Involvement of long bones is also seen, which can cause pathological fractures and growth impairment. There is iron deposition in various organs, which can lead to heart failure or hepatic failure (leading to cirrhosis). Thalassaemias are suspected in presence of family history, or signs suggesting microcytic haemolytic anaemia. Further test and quantitative haemoglobin studies are useful. In beta-thalassaemia, there is an increase in serum bilirubin, iron and ferritin levels. There is severe anaemia, often with haemoglobin < 6 g/dl. There is an elevated red blood cell count, which are microcytic. Peripheral blood smear is diagnostic with nucleated erythroblasts, target cells, small pale red blood cells, and punctate basophilia.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 22
Incorrect
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A 63-year old lady presents with pain in multiple bones and renal failure. On enquiry, there is history of recurrent pneumonia in the past. What will be the likely finding on her bone marrow biopsy?
Your Answer: Granulomas
Correct Answer: Plasma cells
Explanation:Multiple myeloma is a plasma cell malignancy that produce excessive monoclonal immunoglobulins. The disease presents with bone pains, renal dysfunction, increased calcium, anaemia and recurrent infections. Diagnosis is by demonstrating the presence of M-protein in urine or serum, lytic bone lesions, light chain proteinuria or excessive plasma cells on marrow biopsy.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 23
Correct
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Multiple, non-tender lymphadenopathy with biopsy showing several crowded follicles of small, monomorphic lymphocytes and the absence of Reed-Sternberg cells is seen in which of the following?
Your Answer: Poorly differentiated lymphocytic lymphoma
Explanation:Malignant lymphoma usually causes non-tender lymphadenopathy, unlike the tender lymphadenopathy caused by infections (including infectious mononucleosis caused by Epstein-Barr virus). Also, the lymphoid hyperplasia seen in infectious mononucleosis is benign and polyclonal.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 24
Incorrect
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Which of the following is the most likely cause of massive splenomegaly in a 35-year old gentleman?
Your Answer: Haemochromatosis
Correct Answer: Myelofibrosis
Explanation:Causes of massive splenomegaly include chronic myelogenous leukaemia, chronic lymphocytic leukaemia, lymphoma, hairy cell leukaemia, myelofibrosis, polycythaemia vera, sarcoidosis, Gaucher’s disease and malaria.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 25
Correct
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A 65-year old gentleman presents to the clinic with chronic back pain and weight loss. His blood count shows a white blood cell count of 10 × 109/l, with a differential count of 66 polymorphonuclear leukocytes, 7 bands, 3 metamyelocytes, 3 myelocytes, 14 lymphocytes, 7 monocytes, and 5 nucleated red blood cells. The haemoglobin is 13 g/dl with a haematocrit of 38.1%, a mean corpuscular volume of 82 fl, and a platelet count of 126 × 109/l. What is the likely diagnosis?
Your Answer: Metastatic carcinoma
Explanation:The peripheral blood findings suggest a leucoerythroblastic picture, the common causes of which in a 65-year old gentleman includes prostatic or lung malignancy.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 26
Correct
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A 59-year old gentleman admitted for elective cholecystectomy was found to have a haemoglobin 12.5 g/dl, haematocrit 37%, mean corpuscular volume 90 fl, platelet count 185 × 109/l, and white blood cell count 32 × 109/l; along with multiple, small mature lymphocytes on peripheral smear. The likely diagnosis is:
Your Answer: Chronic lymphocytic leukaemia
Explanation:CLL or chronic lymphocytic leukaemia is the most common leukaemia seen in the Western world. Twice more common in men than women, the incidence of CLL increases with age. About 75% cases are seen in patients aged more than 60 years. The blood, marrow, spleen and lymph nodes all undergo infiltration, eventually leading to haematopoiesis (anaemia, neutropenia, thrombocytopenia), hepatomegaly, splenomegaly and decreased production of immunoglobulin. In 98% cases, CD+5 B cells undergo malignant transformation.
Often diagnosed on blood tests while being evaluated for lymphadenopathy, CLL causes symptoms like fatigue, anorexia, weight loss, pallor, dyspnoea on exertion, abdominal fullness or distension. Findings include multiple lymphadenopathy with minimal-to- moderate hepatomegaly and splenomegaly. Increased susceptibility to infections is seen. Herpes Zoster is common. Diffuse or maculopapular skin infiltration can also be seen in T-cell CLL.
Diagnosis is by examination of peripheral blood smear and marrow: hallmark being a sustained, absolute leucocytosis (>5 ×109/l) and increased lymphocytes in the marrow (>30%). Other findings can include hypogammaglobulinemia (<15% of cases) and, rarely, raised lactate dehydrogenase (LDH). Only 10% cases demonstrate moderate anaemia and/or thrombocytopenia.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 27
Incorrect
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Which of the following is the most likely cause of prolonged thrombin clotting time?
Your Answer: Factor VIII deficiency
Correct Answer: Hypofibrinogenemia
Explanation:Thrombin clotting time, also called thrombin time (TT), is test used for the investigation of possible bleeding or clotting disorders. TT reflects the conversion of fibrinogen to fibrin and it’s also very sensitive to the presence of the anticoagulant heparin. A prolonged thrombin time may indicate the presence of hypofibrinogenemia (decreased fibrinogen level ), dysfibrinogenaemia, disseminated intravascular coagulation (DIC), end stage liver disease or malnutrition.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 28
Correct
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A significantly elevated white cell count of 50 x 109/l with 5% blasts and raised leucocyte alkaline phosphatase is seen in which of the following conditions?
Your Answer: Leukaemoid reaction
Explanation:Non-neoplastic proliferation of leucocytes causes an increase in leukocyte alkaline phosphatase (LAP). This is referred to as ‘leukemoid reaction’ because of the similarity to leukaemia with an increased white cell count (>50 × 109/l) with immature forms. Causes of leukemoid reaction includes haemorrhage, drugs (glucocorticoids, all-trans retinoic acid etc), infections such as tuberculosis and pertussis, and as a paraneoplastic phenomenon. Leukemoid reaction can also be seen in infancy as a feature of trisomy 21. This is usually a benign condition, but can be a response to a disease state. Differential diagnosis include chronic myelogenous leukaemia (CML).
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This question is part of the following fields:
- Haematology
- Pathology
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Question 29
Correct
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Which of the following coagulation factors is responsible for the formation of a complex with tissue factor to activate factors IX and X?
Your Answer: Factor VII
Explanation:Factor VII, also known as proconvertin or stable factor, is a vitamin K–dependent protein that plays a central role in haemostasis and coagulation. Tissue factor is a protein that is normally not exposed on the surface of intact blood vessels. Damage to the vascular lumen leads to tissue factor exposure. The exposed tissue factor binds to factor VII. This facilitates the activation of factor VII to factor VIIa.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 30
Incorrect
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Blood investigations of a patient with vitamin K deficiency revealed a prolonged prothrombin time. This coagulation abnormality is most probably due to:
Your Answer: Thrombocytopenia
Correct Answer: Factor VII deficiency
Explanation:Factor VII deficiency is a bleeding disorder caused by a deficiency or reduced activity of clotting factor VII. It may be inherited or acquired at some point during life. Inherited factor VII deficiency is an autosomal recessive disorder caused by mutations of the F7 gene. Factor VII is vitamin K-dependent, as are Factors II, IX and X and therefore lack of this vitamin can cause the development of acquired factor VII deficiency. Other causes of acquired deficiency of this factor include liver disease, sepsis and warfarin therapy.
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This question is part of the following fields:
- Haematology
- Pathology
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SESSION STATS - PERFORMANCE PER SPECIALTY
