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jzbjzb
why not thalassemia major? splenectomy reduces the need for blood transfusions and greater Hb levels
+4
shieldmaiden
Because of the NBME law of the "Best Answer". Splenectomy is what "cures" HS, because that is the organ that prematurely targets it for removal leading to hemolysis. As for thalassemia, you could do it, but it is not indicated at this time for this 10-year-old. Splenectomy is only indicated for thalassemia when an enlarged spleen increased the frequency of transfusions and/or chelation therapy is not enough to control iron levels.
+2
shieldmaiden
For the exam, only consider splenectomy for spherocytosis, refractory autoimmune hemolytic anemia, or massive trauma
+1
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.