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Pediatrics · Haematology: sickle cell disease therapy
Sample itemA 6-year-old with sickle cell anaemia has had three painful vaso-occlusive crises this year. Which drug reduces the frequency of crises by raising fetal haemoglobin?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Hydroxyurea
Hydroxyurea increases HbF, which inhibits polymerisation of HbS, and lowers crises, acute chest syndrome and transfusion needs. Marrow suppression is monitored with blood counts. Folic acid is given for the high red cell turnover but does not reduce crises.
Option by option
A. Folic acid alone
Folic acid supports erythropoiesis but does not reduce crises.
B. Warfarin
Warfarin does not prevent vaso-occlusion.
C. Oral iron
Iron is not needed and may be harmful.
D. Hydroxyurea (correct)
Correct. Hydroxyurea raises HbF and reduces crises.
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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