hsd haematology — Past Paper OmpathStudy

Haematology — February 2021 Specialist Examination Complete transcription of all 8 main questions from all 12 source pages. Subquestions, result tables and case progressions are preserved. The source contains no answer key, so answers have not been invented. Essay paper 1 1. Relate the laboratory investigation of structural red cell disorders to their pathophysiology. 2 2. Provide a broad definition of ‘Benign Ethnic Neutropenia’ (50%) and critically discuss the causes (25%) and clinical implications (25%) of this manifestation. 3 3. Evaluate the causes of pancytopenia and pathways employed for its investigation. 4 4. Briefly describe the pathophysiology of heparin induced thrombocytopenia and critically evaluate the assays available for laboratory detection of the condition. 5 5. Critically discuss the design of assays to diagnose haemophilia, and to monitor treatment of patients with this disorder. Higher Specialist Diploma Haematology Examination - February 2021 Case studies 120 minutes Attempt all case studies Instructions to candidates 1. Record your candidate number and HSD discipline on the front sheet of the answer booklet. 2. Record your candidate number, the question number and the page number in the spaces provided on the answer sheets. 3. Begin each new answer on a new page. 4. Each question is worth 100 marks. Case studies 1 1. A 19-year-old male, originally from India, presents to his GP following two episodes of haematemesis within the previous 24 hours. He reports pallor, lethargy, right upper abdominal pain and progressive abdominal distension. Although he has experienced some degree of distension for ‘the last few years’ he didn’t feel it was necessary to discuss this with his medical practitioner until now. At the age of 13 years, he experienced splenic rupture as a complication for infectious mononucleosis and underwent emergency splenectomy. He reports no history of fever, although has been prone to epistaxis for approximately the last 12 months. Table 1: Initial Full Blood Count Investigations Full Blood count Result Reference range Red cell count (x1012/L) 3.6 4.3-5.7 Haemoglobin (g/L) 98 133-167 Mean Cell Volume (fL) 75 77-98 Mean Cell Haemoglobin (pg) 27 28-40 Platelets (x109/L) 67 143-400 White Blood Cell count (x109/L) 13.4 4.0-10.0 Neutrophils (x109/L) 9.3 2.0-7.0 Lymphocytes (x109/L) 2.8 1.0-3.0 Table 2: Haemostasis investigations Haemostasis Result Reference range Prothrombin Time 13.5 11-14s Activated partial Thromboplastin Time 32.0 24-34 s Table 3: Haematinic investigation Ferritin 520 25-380 μg/ml Figure 1: Initial peripheral blood film A A A Figure 2: Bone marrow aspirate a. Interpret the patient’s full blood count results (Table 1) and clotting results (Table 2). Describe the initial reflex investigation that should be conducted. (10%) b. Figure 1 shows this patient’s peripheral blood film. Identify the features present and indicate, where possible, the likely causes. (10%) A repeated full blood count was requested to confirm the patient’s platelet results (data not shown). Following confirmation of unexplained thrombocytopenia, a bone marrow aspirate was obtained (Figure 2). c. Identify the cells labelled A and indicate the conditions in which cells resembling these may be found. (20%) In order to investigate right upper abdominal pain and distension, an abdominal ultrasound and CT were requested. The abdominal ultrasound confirmed hepatomegaly whilst subsequent abdominal CT indicated portal hypertension. Given these findings, a transjugular liver biopsy was performed and revealed centrilobular and portal fibrosis with large numbers of diffusely spread Kupffer cells. Given this additional information: d. Explain the association between these most recent findings and haematemesis. (20%) Consequent of the clinical picture and laboratory findings, a β-glucosidase assay was performed. The result was 0.75 (RR in leucocytes 1.0-5.0 nmol/mg/hr). e. Explain the significance of these findings and provide an exact diagnosis for this patient. (20%) f. Describe the patient’s ferritin results (table 3) and discuss these in the context of the patient’s blood cell picture and diagnosis. (10%) In patients diagnosed with this condition, it is recommended that baseline immunoglobulin levels are determined and monitored once every two years for patients below 50 years of age and once per annum in those aged over 50 years. g. Explain the basis of this recommendation. (10%) UNSEEN CASE STUDIES 2 2. A 50 year old male, originally from Iran, presented to A&E with shortness of breath and was discovered to have severe anaemia. He was previously fit and well but on taking a detailed history, he described a similar episode of severe anaemia 15 years previously. He spent one week as a hospital inpatient and was discharged with further outpatient visits planned on the 6th April and 8th May. His results over this 4 month period were as follows: Haematology & Blood Transfusion A/E In-patient Haem Clin