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Part 1 examination Haematology First paper Tuesday 24 September 2019 Candidates must answer ALL questions Time allowed: Three hours Question 1 - Transfusion A 54 - year-old male with Hb SS with recurrent crisis and history of acute chest syndrome several years and pulmonary hypertension, has been admitted following a mechanical fall. X-ray findings confirm fracture right neck of femur. He needs urgent orthopaedic surgery but is reluctant to agree to have blood transfusion as he is concerned about the safety of blood and his wife is a Jehovah’s Witness. He is blood group B RhD negative. His Hb S was 84% when last checked 4 months ago and Hb at admission was 90 g/L. a) Discuss how you will manage his concerns and what you need to do to optimise him for surgery. Discuss potential complications in this patient including those related to treatment. (20 marks) b) Describe the specification of blood components, volume required for exchange transfusion and targets for treatment. (5 marks) Question 2 - Haemato-oncology A previously fit and healthy 62 year old man presents with fatigue and back pain and is found to have a Hb of 82g/l and Creatinine of 350umol/l. A diagnosis of multiple myeloma is suspected: a) Discuss the initial investigations and urgent acute management that is required. ( marks) b) What are the recommended options for imaging in newly diagnosed patients with myeloma? (4 marks) c) Discuss the options for therapy for this man and the factors that would guide your decisions. ( marks) d) 28 months later, he then presents with rapid onset pain and weakness in his left leg associated with urinary incontinence. Discuss the likely diagnosis and initial plans for management in this situation. (5 marks) Question 3 - Haemostasis A 74-year-old man underwent a complicated femoral-popliteal bypass as is started on unfractionated heparin post-operatively. a) What clinical situations is unfractionated heparin used in preference to low molecular weight heparins. ( marks)
b) List three methods for monitoring the effect of unfractioned heparin and describe one of these methods in detail discussing the advantages and disadvantages of it ( marks) c) The patient needs to go back to theatre in the next few hours as there are worries over the patency of the graft, describe how the effect of unfractionated heparin can be reversed and compare this to the reversal of low molecular weight heparin. (8 marks) Question 4 - General Haematology A 35 - year-old man referred urgently with Hb 210g/L. The patient had been attending a gym and had an episode of lightheadedness. a) What is your advice to the GP and management plan? (10 marks) 4 weeks later he is in Haematology clinic with a negative Jak2 result. b) What further investigations? Discuss ongoing management (15 marks)
Question 3 – Haem-oncology A 60 year old man who was previously fit and well presents to his GP with a three month history of neck swelling accompanied by fatigue, lethargy and loss of 10kg of weight. On examination he had lymphadenopathy in all cervical stations, the largest being 3cm in diameter. A CT scan showed disease in the axillae, mediastinum and retroperitoneum, as well as 18 cm splenomegaly. He was referred to an ENT surgeon for a biopsy. The pathology is presented at your MDT and reveals a diagnosis of mantle cell NHL. a) Describe the pathological and immunophenotypical features you would expect to see. Discuss your investigation and management plan for this patient. How would you explain this to the patient? (50%) b) 36 months later at a routine clinic appointment he reports recurrence of his presenting symptoms and has clinical evidence of relapse with widespread lymphadenopathy and hepatosplenomegaly. What are the options for further systemic anticancer treatment now and which is your preferred treatment? Briefly describe the mode of action of any treatment you might use and any side effects that you would look out for. (35%) c) 12 months later he relapses again with no B symptoms and palpable left axillary nodes only. How would you treat him now? (15%) Question 4 – Transfusion You are contacted by the hospital transfusion laboratory at 2 am to say that the transplant team are about to perform a heart transplant on a 44 year old man, but he has a positive direct antiglobulin test (DAT) and the red cell antibody panel is pan- reactive. a) What are the possible causes of the positive DAT and what laboratory investigations will help you decide on the cause? (50%) b) What is the differential diagnosis for this patient? (30%) c) How would you manage the provision of red cell support? (20%)
2. Haemostasis and thrombosis You are asked to see in clinic a 29 year old woman at the request of the obstetric department. She has a history of 3 consecutive first trimester miscarriages and an anatomical cause has been excluded. Investigations have shown a normal haemoglobin, white count and platelet count and PT and fibrinogen, but the APTT is prolonged 15 seconds beyond the upper limit of normal. a) Discuss in general the differential diagnosis of an isolated prolonged APTT and the history and laboratory investigations you would perform to clarify the cause. (15 marks) b) Describe the clinical and laboratory features and the criteria for diagnosis of anti- phospholipid syndrome. **(10 marks)
Question 3: Haematological Oncology A 60 year old man presents with drowsiness, nosebleeds, headaches and blurred vision. On examination he has retinal haemorrhages, 4 fingerbreadths of splenomegaly and extensive 1 - 2 cm lymphadenopathy. Blood count shows Haemoglobin 78 g/L, platelets 76 x10^9 /L, and white blood cell count 1.5 x 10^9 /L. His serum total protein is elevated, and total IgM is raised at 28 g/L with a paraprotein demonstrated on serum electrophoresis. A bone marrow trephine biopsy reveals dense infiltration with lymphoplasmacytoid lymphocytes. a) What is the diagnosis? What further investigations are required to identify the cause of his presenting symptoms and clarify the underlying problem? Indicate the appropriate initial management of the patient (7 marks) b) With appropriate initial management, his symptoms improve significantly. What options for systemic anti-cancer treatment would you offer him to treat the underlying condition? Describe what aims of treatment and potential adverse effects you would discuss with the patient in order to gain written informed consent for the proposed treatment (9 marks) c) He responds well to first line chemotherapy and achieves a remission. 12 months later he represents with renewed nosebleeds and a significant rise in the IgM paraprotein. What is the appropriate management at this stage? Are there any molecular genetic tests that might guide your choice of therapy? (9 marks) Question 4: Haemostasis and Thrombosis You are asked for advice on a 70 year old woman with a platelet count of 45 x 10^9 /L who had undergone coronary artery bypass graft surgery 6 days previously. Full blood count and coagulation screen (prothrombin time and activated partial thromboplastin time) are otherwise normal. The blood film confirms the thrombocytopenia is genuine with no other abnormalities seen and review indicates that the preoperative platelet count was normal. a) Discuss the differential diagnosis and any further history you will obtain to help establish the diagnosis (7 marks) b) Outline the pathophysiological basis of heparin induced thrombocytopenia (HIT). Discuss the laboratory tests used to investigate suspected cases of HIT and how the findings of these may be interpreted in its diagnosis (12 marks) c) Outline the anticoagulation options available for the treatment of HIT including the duration of treatment and describe any factors that may influence your choice of treatment. (6 marks)
Question 3: Transfusion medicine A blood sample sent to the hospital blood bank for crossmatching is found to have a positive direct antiglobulin test (DAT). What are the possible causes of the positive DAT, what further features in the history and laboratory investigations would be appropriate and how would you manage the provision of red cell support? Question 4: General Haematology A GP seeks advice on a 23 year old patient with (haemoglobin) Hb SS, estimated 10 weeks pregnant. She is para 0 + 0. She wants to know if she is going to have an affected child & what antenatal care she should have. a) Outline the detail of the investigations that you would recommend the GP to send to the laboratory prior to the patient being seen in 1 weeks time in clinic b) List the relevant tests for her partner and the rationale for these c) Draw up an outline of how you would approach care in pregnancy with reference to potential complications
Question 1: Haemostasis and thrombosis You are asked to see a 35 year old female in clinic who requires tonsillectomy. She has been referred from pre-op assessment clinic having reported “bleeding” following a previous dental extraction. Discuss features of the clinical history and examination that would be relevant. Which investigations you would subsequently request – please explain why you would perform these investigations and any caveats in the interpretation of results. Question 2: Transfusion Describe the strategies used to keep the blood supply free from potential viral infections, using 2 example viruses (include one virus recognised as an issue in the past 3 years) to illustrate your answer. What practices, other than strategies to minimise the risk of viral contamination of a product, maximise clinical safe and effective blood transfusion for patients?