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MB BCh III / GEMP I INTEGRATED BASIC MEDICAL AND HUMAN SCIENCES (SCMD 3000)
Venue: Multipurpose Path Lab (3N28) Seat Number: ___________ Student Number: _________________________________ Time allowed: 90 minutes
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GENERAL INSTRUCTIONS Please read the following carefully: 1. Write your session number, seat number, student number and surname & Initials clearly on the front page of this question paper (top and middle of page). 2. Then write your initials and surname at the top of each page of this exam paper. 3. Please hand in all cell phones, calculators and removable drives prior to the start of the examination. 4. You may not remove any question papers from the examination room. 5. This paper consists of 45 multiple choice questions 6. Answer all questions on the script provided AND THEN ON THE ANSWER CARDS PROVIDED. A-TYPE QUESTIONS Select the single best answer to each question. (Note: several options may be correct but only one is the best answer.) Answers must be entered on the question paper and on the answer card for A-type questions. If you give more than one answer for a question, you will score zero for that question. There will be no penalty for incorrect answers. If you do not know an answer you may leave it blank, in which case you will neither earn a mark nor be penalised.
X-TYPE QUESTIONS There is at least one correct statement and at least one incorrect statement. Identify BOTH the correct and incorrect statements. Answers must be entered on the question paper and on the answer card for X-type questions. If you are unsure of a statement you can leave the answer bubbles blank or you can tick the Dont know box. In that case you will neither get a mark or be penalised. Negative marking will be applied to wrong answers but negative marks will not be carried forward. This means the minimum mark for a question is zero.
R-TYPE QUESTIONS Here you are offered a number of answers. This is followed by a set of questions. Select the single best answer to each question. Note that each answer may be used more than once or not at all unless you are specifically instructed otherwise. Fill in your answers in the spaces provided on the question paper and on the answer card for R-type questions. If you do not know an answer you may leave it blank, in which case you will neither earn a mark nor be penalised.
Note: Each A-type, X-type and R-type question has the same mark value except where indicated otherwise.
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A-TYPE QUESTIONS
A.1
James and Josh have Haemophilia A. Kate is 18 weeks pregnant with a male fetus. What is the chance, prior to any testing, that the fetus will be affected with Haemophilia A? a. b. c. d. e. 1 in 2 (50%) 1 in 4 (25 %) 1 in 8 (12.5 %) 1 in 16 (6.25%) No chance that she will have an affected child
Answer: b Chance that Kate is a carrier x Chance that she passes it on = (very easy) A.2 Loss of tumor suppression in a cell usually results from one of the following. Which one? a. b. c. d. e. Answer: b A.3 Chronic granulomatous disease (CGD) is due to one of the following primary immune deficiency conditions. Which one? a. b. c. d. e. Answer: a A defect in neutrophil function such as an absence of NADPH oxidase Hypocomplementaemia Neutrophil phagosome-lysosome fusion Reduced B cell function Reduced T cell numbers Activation of a proto-oncogene by a virus A mutation of both copies of a tumor suppressor gene An inversion involving a copy of a tumor suppressor gene Cytokine activation of a tumor suppressor gene Over expression of a tumor suppressor gene due to a translocation
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X-TYPE QUESTIONS
X.1
A group of diseases is characterised by the presence of the cell in the image above. Which of the following statements about these diseases are correct, and which are not? a. b. c. d. e. Involvement of lymph nodes on both sides of the diaphragm constitutes Stage III disease Spread of the disease is contiguous and orderly from one lymph node group to the next The classification takes into account the number and type of NON-MALIGNANT cells present The presence of B symptoms indicates a better prognosis The prognosis is poor, even in early stage disease
Answer: a, b, c X.2 Which of the following pairs are correctly matched with respect to B lymphocyte development, and which are not? a. b. c. d. e. germinal centre centroblasts (large cells) AND undergoing somatic hypermutation germinal centre centrocytes (small cells) AND resistant to apoptosis initial rearrangement of heavy and light chain genes AND occurs in the bone marrow marginal zone lymphocytes AND unexposed to antigen (nave B cells) plasma cells AND surface expression of antibody
Answer: a, b, c X.3 Which of the following pairs are correctly matched with respect to Non-Hodgkins lymphomas, and which are not? a. Adult T cell lymphoma AND caused by the HTLV-1 retrovirus (HTLV-1: human Tlymphotropic virus) b. Burkitts lymphoma AND t(8;14) involving myc and the immunoglobulin heavy chain gene c. Diffuse large B cell lymphoma AND slow growing lymph nodes d. Follicular lymphoma AND treated with antibodies to B cell antigens e. Mycosis fungoides AND B cell lymphoma involving skin Answer: a, b, d
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X.4
The above peripheral blood comes from a 68 year old patient with a white cell count of 75 x 109/l. Which of the following statements with respect to this disease are correct, and which are not? a. b. c. d. e. Cytogenetic markers have no prognostic significance Development of microcytic, hyperchromic anaemia is a well-known complication This patient is at increased risk for bacterial infections This patient is likely to have significant lymphadenopathy This patients age is unusual as this is a disease of young people
Answer: b, c, d
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B.
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D.
X.5
E.
Which of the following changes in the differential count are correctly matched with the cells labelled A to E above, and which are not? a. b. c. d. e. Cell A Cell B Cell A Cell D Cell C AND AND AND AND AND increased due to increased marrow production in corticosteroid therapy increased in chronic bacterial infections increased in acute viral infections decreased in corticosteroid therapy increased in pertussis (whooping cough)
Answer: b, d, e X.6 In chronic myeloid leukaemia (CML): a. b. c. d. e. The cell of origin is more differentiated than the CFU-GEMM cells The BCR-ABL protein phosphorylates a number of different substrates in the cytoplasm Untreated people usually die of marrow failure caused by fibrosis of the marrow Imatinib resistance may develop after an initial good response The raised white count is due to maturation arrest of neutrophils
Answer: b, d X.7 Which of the following statements regarding the myeloproliferative neoplasms are correct, and which are not? a. Essential thrombocythemia may present with abnormal bleeding b. Expansion of haemopoiesis occurs in sites of fetal haemopoiesis c. Primary myelofibrosis has an initial proliferative phase with elevated neutrophil and/or platelet counts. d. Proliferation of red cells in Polycythemia vera is dependant on erythropoietin e. Transformation to acute leukaemia is the usual terminal event for patients with Essential thrombocythemia Answer: a, b, c
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Answer: b, c, d X.10 Which of the following statements regarding the management of venous thrombosis are correct, and which are not? a. Heparin therapy in DVT has the direct effect of shrinking the clot. b. If the patient has a prolonged PTT and PT which do not correct, an antiphospholipid antibody should be suspected. c. Low molecular weight heparin (LMWH) has a higher incidence of bleeding complications than unfractionated heparin. d. The patient should be fully anticoagulated with heparin (unfractionated or LMWH) before starting warfarin therapy. e. When using unfractionated heparin, the aim is to increase the baseline PTT by a factor of 5 (5 x increase). Answer: b, d
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Answer: a, c, d X.13 Which of the following statements regarding iron absorption, transport and storage are correct, and which are not? a. b. c. d. e. Hepcidin inhibits the release of iron by cells Iron is absorbed in the intestinal lumen by the divalent metal transporter DMT1 Iron stores are regulated by the secretion of iron Most of the iron in the body is in the form of hepatic enzymes Transferrin bound to iron attaches to the transferrin receptor on the cell surface
Answer: a, b, e X.14 Which of the following statements about iron deficiency are correct, and which are not? a. It causes an increase in transferrin levels b. It causes a reduction in red cell size before it leads to hypochromia c. It characteristically leads to the presence of acanthocytes in the blood d. It is best diagnosed biochemically by a low serum iron concentration e. Without an obvious dietary cause or source of bleeding in an adult should lead to investigation for occult GIT blood loss
Answer: a, b, e
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Answer: a, c, e X.16 Which of the following are correctly matched with respect to pancytopaenia, and which are not? a. b. c. d. e. Acute lymphoblastic leukaemia AND more common in children than in adults Aplastic anaemia AND underlying cause unknown in many cases Hypersplenism AND reduced reticulocyte production index (RPI) Marrow infiltration by carcinoma AND presence of teardrop red cells Megaloblastic anaemia AND hypocellular bone marrow
Answer: a, b, d X.17 Which of the following statements regarding sickle cell anaemia (homozygous) are correct, and which are not? a. b. c. d. e. Chronic organ damage results from repeated episodes of infarction Hydroxyurea is used to increase the HbF concentration Option Removed It protects against severe malaria The red cells sickle in hypoxic conditions
Answer: a, b, e X.18 Which of the following statements regarding the thalassaemias is/are correct, and which is/are not? a. thalassaemia presents at birth b. In thalassaemias involving deletions of the globin gene cluster on chromosome 11, there may be loss of the and genes c. Iron overload is a major cause of morbidity and mortality in thalassaemia major d. Patients with 0 thalassaemia major are able to produce small quantities of globin chains e. The chain precipitation leads to intramedullary haemolysis Answer: b, c, e
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Answer: b, c, e X.20 Which of the following are correctly matched with respect to non-haemolytic transfusion reactions, and which are not? a. b. c. d. e. Graft versus host disease AND less common in related host-donor pairs Febrile non-haemolytic transfusion reactions AND Antibodies to donor leucocytes Post transfusion purpura AND destruction only of donor platelets Recipient sensitised to protein in donor blood AND urticaria Transfusion-related acute lung injury (TRALI) AND clinically indistinguishable from ARDS (adult respiratory distress syndrome)
Answer: b, d, e X.21 In which of the following settings does polycythaemia occur? a. b. c. d. e. Chronic obstructive airways disease Increased binding of carbon monoxide to Hb in smokers Left to right shunts in congenital heart disease Mutations of Hb leading to low O2 affinity Secretion of erythropoietin in renal disorders such as hydronephrosis
Answer: a, b, e X.22 Which of the following statements regarding platelet disorders are correct, and which are not? a. Acute idiopathic thrombocytopenic purpura (ITP) is more common than chronic ITP in adults b. Alcohol suppresses megakaryocyte synthesis c. In Bernard-Soulier syndrome there is a deficiency of Glycoprotein 1b (GPIb) d. In Glanzmans disease the activated platelets are unable to aggregate e. The effect of aspirin on platelets has a half life of 8 hours Answer: b, c, d
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Answer: a, c, d X.24 Which of the following statements regarding von Willebrands factor (vWF) and von Willebrands disease (vWD) are correct, and which are not? a. b. c. d. e. vWF is necessary for adhesion of the platelet via Glycoprotein IIb IIIa vWF is necessary for the stabilization of factor VIII vWF is produced in the endothelium Type II vWD is usually due to gene deletion Type IIb vWD is a cause of thrombocytopenia
Answer: b, c, e X.25 With respect to anti-platelet drugs: which of the following are correctly paired, and which are not? a. b. c. d. e. Cyclooxygenase : Clopidogrel P2Y12 receptor : Aspirin Phosphodiesterase inhibition : Ridogrel RGD sequence : Integrelin Thromboxane synthetase : Dazoxiben
Answer: d, e
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Answer: a, c, X.27 Which of the following statements about drugs used in the treatment of leukaemia are correct, and which are not? a. b. c. d. e. Cladribine is used to treat chronic B cell lymphocytic leukaemia Concurrent use of rifampicin increases the plasma concentration of imatinib Dasatinib is used as an alternative in imatinib resistance Methotrexate inhibits thymidylate synthase Rituximab causes a selective depletion of T-cells
Answer: a, c, d b: Rifampicin induces metabolism of imatinib thus reduces plasma concentration c: Correct used in cases of imatinib resistance d: Mechanism of action of methotrexate X.28 Which of the following statements bout anticoagulant and fibrinolytic agents are correct, and which are not? a. b. c. d. e. abciximab prevents the binding of fibrin to GPIIb/IIIa T enoxaparin is contraindicated in the treatment of deep vein thrombosis heparin increases the affinity of antithrombin III for thrombin T streptokinase is used to prevent clotting after major surgery warfarin-induced haemorrhage is rapidly reversed by withdrawing the drug
Answer: a, c b: enoxaparin is used to treat d: streptokinase is contraindicated e: freshly frozen plasma is used drug withdrawal too slow X.29 Which of the following statements regarding the immune system are correct, and which are not? a. b. c. d. e. Adhesion molecules such as ICAM1, LFA1 and LFA3 are present on all leukocytes Cytotoxic T cells express CD3 and CD4 molecules Iinterleukin 2 is produced by macrophages Th1 cells produce histamine The adaptive response is due to T and B cell memory
Answer: a, e
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R-TYPE QUESTIONS
A. B. C. D. E. F. G. H. Liver failure Warfarin treatment Unfractionated heparin therapy Antiphospholipid syndrome Haemophilia A Disseninated intravascular coagulation (DIC) Haemophilia B Von Willebrands disease
The following are coagulation screen results for hospitalised patients who are bleeding from their drip sites. Choose the correct diagnosis of causes of bleeding from the list above. Parameter PT Correction PTT Correction Fibrinogen D-dimers Factor V Factor VIII Factor XII Factor VII Factor X Factor II Antithrombin Platelets R.3 Patient 3 Normal range 12 16 seconds 23 39 seconds 2-4 <0.2mg/ml 50 150% 50 150% 50 150% 50 150% 50 150% 50 150% 75 125% 175 450 x 109/l Patient 1 25 14 83 32 1.5 0.2 42 49 28 25 32 33 70 27 Patient 2 24 13.5 75 33 1.8 4.5 45 115 32 20 25 28 35 180 Patient 3 80 14.2 119 38 3.8 0.2 72 149 65 4 7 10 120 365
Answer: B
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Answer: E R.5 A 48 year old woman with pallor and mild jaundice is taking thyroxine for autoimmune thyroiditis. Her FBC is as follows: Parameter Result Normal range WCC 2.2 4.00 10.00 x109/l RCC 1.13 4.13 5.67 Hb 4.2 12.1 16.3 Hct 0.13 0.37 0.49 MCV 115 79.1 98.9 fl MCH 37 27 32 pg MCHC 32 32 36 g/dl RDW 25 11.6 14 % Platelets 36 137 373 x109/l
Answer: B