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NBME 17 Answers

nbme17/Block 4/Question#47 (8.7 difficulty score)
A 10-year-old boy has had anemia since birth. ...
Hereditary spherocytosisπŸ”

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submitted by cassdawg(959),

Splenectomy is indicated for hereditary spherocytosis because it is an intrinsic hemolytic anemia where the spleen is destroying the red cells even though they could technically function fine. Thus splenectomy will prevent premature removal by the spleen (FA2020 p422).

Sickle cell disease causes autosplenectomy/splenic infarct/sequestration but splenectomy is not indicated because the spleen is not contributing to the symptoms of sickle cell, the symptoms are caused by the vasoocclusive disease.