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General Surgery · Spleen: hereditary spherocytosis
Sample itemA 7-year-old child with hereditary spherocytosis has persistent haemolysis and transfusion requirements. What is the treatment of choice?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Splenectomy after age 5 to 6 years
Splenectomy removes the site of destruction of the fragile spherocytes and cures most of the anaemia, though spherocytes persist. It is delayed beyond about 5 to 6 years to lower infection risk. Concomitant cholecystectomy is performed if gallstones are present.
Option by option
A. Splenectomy after age 5 to 6 years (correct)
Correct. Splenectomy after early childhood is the standard.
B. Lifelong steroids
Steroids are not useful in this inherited defect.
C. Splenectomy in the first year of life
Very early splenectomy raises infection risk.
D. Bone marrow transplantation as first-line
Transplantation is not first-line.
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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