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General Medicine · Haematology: thalassaemia major
Sample itemA 2-year-old child has severe anaemia, hepatosplenomegaly and frontal bossing. Haemoglobin electrophoresis shows predominantly HbF. What is the cornerstone of management?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Regular transfusions with iron chelation
Beta thalassaemia major presents in infancy with transfusion dependence and extramedullary haematopoiesis. Regular transfusions maintain haemoglobin above 9 to 10 g/dL and chelation with deferasirox or deferiprone prevents iron overload. Bone marrow transplantation is curative.
Option by option
A. Splenectomy at diagnosis
Splenectomy is for hypersplenism.
B. Regular transfusions with iron chelation (correct)
Correct. Transfusion with chelation is standard.
C. Folic acid alone
Folic acid alone is inadequate.
D. Iron supplementation
Iron supplements worsen overload.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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