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Pediatrics · Haematology
Sample itemA 2-year-old with severe anaemia needing regular transfusions since infancy has frontal bossing and hepatosplenomegaly. A skull radiograph shows a hair-on-end appearance. Which therapy is needed to prevent complications of the treatment?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Iron chelation therapy
Beta-thalassaemia major causes ineffective erythropoiesis, marrow expansion, and the hair-on-end skull. Lifelong transfusion causes iron overload, damaging heart, liver and endocrine glands. Chelators such as deferasirox or desferrioxamine are used, and hydroxyurea or bone marrow transplantation may be used in select cases.
Option by option
A. Oral iron supplements
Oral iron worsens iron overload in transfusion-dependent patients.
B. Vitamin B12 injections
Vitamin B12 deficiency is not the cause.
C. Corticosteroids
Corticosteroids do not treat thalassaemia.
D. Iron chelation therapy (correct)
Correct. Chelation removes transfusional iron and prevents cardiac and endocrine damage.
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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