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Question 1
Incorrect
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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: Previous splenectomy
Correct 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 2
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: Disseminated intravascular coagulopathy
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 3
Correct
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A 50-year old lady presented to the clinic with chronic pain in the abdomen. On physical examination, she was found to be pale. Further investigations revealed a decrease in both serum iron and total iron-binding capacity, along with an increase in serum ferritin. These findings are seen in:
Your Answer: Anaemia of chronic disease
Explanation:Anaemia of chronic disease is characterized by low serum iron, iron-binding capacity and saturation with increased ferritin (storage iron). Haemolytic anaemia is characterized by normal iron levels as the haemoglobin released from the haemolysed red blood cells is recycled. Anaemia due to chronic blood loss leads to low serum iron, low ferritin and high total iron-binding capacity (TIBC). Malabsorption, especially with duodenal involvement can also lead to iron deficiency anaemia with low ferritin and high TIBC. Megaloblastic anaemia due to vitamin B12 and folate deficiency is not associated with abnormalities in metabolism of iron.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 4
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:
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 5
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:
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 6
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:
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 7
Incorrect
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A 30-year-old woman known with Von Willebrand disease (vWD) has to undergo surgery. Which of these complications is most unlikely in this patient?
Your Answer:
Correct Answer: Hemarthrosis
Explanation:Von Willebrand disease (vWD) is an inherited haemorrhagic disorder characterised by the impairment of primary haemostasis. It is caused by the deficiency or dysfunction of a protein named von Willebrand factor. The most common manifestation due to the condition is abnormal bleeding. Complications include easy bruising, hematomas, epistaxis, menorrhagia, prolonged bleeding and severe haemorrhage. Hemarthrosis is a complication that is more commonly found in haemophilia.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 8
Incorrect
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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:
Correct 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 9
Incorrect
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Question 10
Incorrect
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A Monospot test in a 17-year old boy presenting with fever, multiple palpable lymph nodes and mild icterus was positive. His blood investigation is likely to show which of the following?
Your Answer:
Correct Answer: Atypical lymphocytosis
Explanation:Epstein-Barr virus is the causative agent for infectious mononucleosis leading to presence of atypical lymphocytes in blood. Usually symptomatic in older children and adults, the incubation period is 30-50 days. Symptoms include fatigue, followed by fever, adenopathy and pharyngitis. Fatigue can last for months and is maximum in first few weeks. Fever spikes in the afternoon or early evening, with temperature around 39.5 – 40.5 °C. The ‘typhoidal’ form where fatigue and fever predominate has a low onset and resolution. Pharyngitis resemble that due to streptococcus and can be severe and painful. Lymphadenopathy is bilaterally symmetrical and can involve any nodes, specially the cervical ones. Mild splenomegaly is seen in 50% cases, usually in 2-3rd week. Mild tender hepatomegaly can occur. Less common manifestations include maculopapular eruptions, jaundice, periorbital oedema and palatal enanthema. Diagnostic tests include full blood count and a heterophil antibody test. Morphologically abnormal lymphocytes account for 80% cells and are heterogenous, unlike leukaemia.
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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 conditions is likely to result in splenomegaly, hypochromic anaemia and hemochromatosis in a young male?
Your Answer:
Correct 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 12
Incorrect
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A patient presented with continuous bleeding several hours after dental extraction. Which of the following findings is most often associated with clinical bleeding?
Your Answer:
Correct Answer: Factor IX deficiency
Explanation:Factor IX deficiency, also called Haemophilia B or Christmas disease, is a disorder caused by missing or defective clotting factor IX. Deficiency of the factor IX causes irregular bleeding that can happen spontaneously or after mild trauma, surgery and dental extractions.
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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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Question 14
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:
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 15
Incorrect
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A 58-year-old woman diagnosed with deep vein thrombosis had been using warfarin for 10 days. When she presented to the doctor she had haemorrhagic bullae and necrotic lesions in her lower limbs and buttocks. Deficiency of which of the following proteins may have caused the necrotic skin lesions?
Your Answer:
Correct Answer: Protein C
Explanation:Warfarin-induced skin necrosis is a rare complication of anticoagulant therapy that requires immediate drug cessation. The most common cutaneous findings include petechiae that progress to ecchymoses and haemorrhagic bullae. Warfarin inactivates vitamin K-dependent clotting factors II, VII, IX, and X and vitamin K-dependent proteins C and S. The concentration of protein C falls more rapidly than other vitamin K-dependent factors because they have a shorter half-lives. Skin necrosis is seen mainly in patients with prior protein C deficiency.
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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 14 year-old girl is found to have haemophilia B. What pathological problem does she have?
Your Answer:
Correct 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 17
Incorrect
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The absence of which of the following components characterizes the grey platelet syndrome (GPS)?
Your Answer:
Correct 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 18
Incorrect
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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:
Correct 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 19
Incorrect
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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:
Correct 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 20
Incorrect
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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:
Correct 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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SESSION STATS - PERFORMANCE PER SPECIALTY
