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Pediatrics · Systemic paediatrics: thalassaemia major transfusion
Sample itemA 2-year-old with transfusion-dependent beta thalassaemia major is started on regular transfusions. What pre-transfusion haemoglobin target and curative option apply?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. 9.5 to 10.5 g/dL; haematopoietic stem cell transplantation
Transfusion regimens maintain a pre-transfusion haemoglobin of 9.5 to 10.5 g/dL to suppress ineffective erythropoiesis, with iron chelation to prevent overload. Allogeneic stem cell transplantation is the only established cure.
Option by option
A. 11 to 12 g/dL; folic acid alone
Folic acid alone is inadequate.
B. 6 to 7 g/dL; splenectomy
Under-transfusion leads to bone deformities and iron absorption.
C. 9.5 to 10.5 g/dL; haematopoietic stem cell transplantation (correct)
Correct. This is the standard target and cure.
D. 13 to 14 g/dL; iron supplements
Iron supplements are contraindicated.
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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