Haematology 10 Single Best Answer (SBA)
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1. What is the primary mechanism underlying the neurological symptoms of vitamin B12 deficiency?
Answer: Disruption of myelin synthesis due to methylmalonic acid accumulation
Vitamin B12 deficiency leads to elevated methylmalonic acid, which interferes with fatty acid synthesis in myelin sheaths, resulting in neurological symptoms.
2. A 40-year-old woman presents with fatigue and pallor. Blood tests show low haemoglobin, low MCV, and low ferritin. What is the most appropriate next investigation?
Answer: Coeliac disease screen
Iron deficiency anaemia in younger women can be due to diet, menstrual losses, or malabsorption (e.g., coeliac disease). Screening is important to exclude it.
3. What is the most common long-term complication of sickle cell disease?
Answer: Chronic kidney disease
Sickle cell-related renal medullary damage eventually leads to chronic kidney disease in many patients.
4. A 25-year-old woman presents with prolonged bleeding after dental surgery and menorrhagia. Von Willebrand factor (VWF) levels and ristocetin cofactor activity are low. What is the most likely diagnosis?
Answer: Von Willebrand's disease type 1
The most common form of vWD is type 1, involving a partial quantitative deficiency of von Willebrand factor.
5. A 65-year-old man presents with incidental thrombocytosis on a full blood count. JAK2 mutation testing is positive. What is the most likely diagnosis?
Answer: Essential thrombocythaemia
In essential thrombocythaemia, clonal proliferation of megakaryocytes leads to high platelet counts, often with a JAK2 mutation.
6. A 30-year-old woman with beta-thalassaemia major asks about pregnancy planning. What is the most important advice?
Answer: Genetic counselling for partner screening
In beta-thalassaemia major, partner screening via genetic counselling helps ascertain risk of having a child with severe thalassaemia.
7. A 70-year-old man presents with bone pain and fatigue. Serum protein electrophoresis shows an M spike, and urine tests detect Bence Jones proteins. What is the most likely diagnosis?
Answer: Multiple myeloma
Multiple myeloma is characterised by clonal plasma cell proliferation, M protein on electrophoresis, and often Bence Jones proteinuria.
8. What is the primary pathophysiological abnormality in polycythaemia vera?
Answer: Clonal proliferation of red blood cells due to JAK2 mutation
Polycythaemia vera is a myeloproliferative neoplasm driven by a JAK2 mutation causing excessive RBC production.
9. A 65-year-old woman develops fever, hypotension, and back pain during a blood transfusion. What is the most appropriate initial management?
Answer: Stop the transfusion immediately and administer IV fluids
In a suspected transfusion reaction, the priority is stopping the transfusion, supportive care (e.g., IV fluids), and investigating for haemolysis or infection.
10. A 22-year-old man with sickle cell disease refuses blood transfusion despite severe anaemia and chest pain suggestive of acute chest syndrome. What is the most appropriate next step?
Answer: Respect his decision and provide supportive care
If a patient with capacity refuses a lifesaving treatment, that choice must be respected, while offering all other supportive measures.
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