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Question 1
Incorrect
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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 VIII
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 2
Incorrect
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Which of the following is the most likely cause of prolonged thrombin clotting time?
Your Answer: von Willebrand factor 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 3
Correct
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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: 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 4
Incorrect
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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 40-year-old man with peripheral basophilia noted on a peripheral blood smear
Correct 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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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: 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 6
Incorrect
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Which of the following coagulation factors cross-links fibrin?
Your Answer: Factor XII
Correct Answer: Factor XIII
Explanation:Factor XIII, also known as fibrin stabilizing factor, is an enzyme of the coagulation cascade that crosslinks fibrin. Deficiency of FXIII may cause bleeding tendency but paradoxically, it may also predispose to thrombosis.
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This question is part of the following fields:
- Haematology
- Pathology
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Question 7
Correct
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A Jewish man was diagnosed with haemophilia C. Which of the following factors is deficient in this form of haemophilia?
Your Answer: Factor XI
Explanation:Haemophilia C, also known as plasma thromboplastin antecedent (PTA) deficiency or Rosenthal syndrome, is a condition caused by the deficiency of the coagulation factor XI. The condition is rare and it is usually found in Ashkenazi Jews.
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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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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: Infectious mononucleosis
Correct 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 9
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 10
Incorrect
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Question 11
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 12
Correct
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Question 13
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: Mild thrombocytopenia
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 14
Correct
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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: 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 15
Incorrect
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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 lymphoblastic leukaemia (ALL)
Correct 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 16
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 17
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 18
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: Factor XII deficiency
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 19
Correct
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Laboratory findings in a patient with dark urine and yellowish skin revealed a prolonged prothrombin time. Which of the following is the most likely cause of this finding?
Your Answer: Liver damage
Explanation:Various conditions may prolong the prothrombin time (PT), including: warfarin use, vitamin K deficiency, liver disease, disseminated intravascular coagulopathy, hypofibrinogenemia, heparin infusion, massive blood transfusion and hypothermia. Liver disease causes prolonging of PT due to diminished synthesis of clotting factors. Dark urine colour and jaundice are indicators of the presence of a liver disease in this patient.
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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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Which of the following is the most likely cause of massive splenomegaly in a 35-year old gentleman?
Your Answer: Portal hypertension
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 21
Correct
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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: 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 22
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:
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 23
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 24
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:
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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Question 25
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 26
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:
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 27
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 28
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 29
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 30
Incorrect
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Which of the following proteins acts as cofactor in the thrombin-induced activation of anticoagulant protein C?
Your Answer:
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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