Abstract
The rationale for splenectomy in blood dyscrasias of children is based on the removal of an organ in which gross aberrations of one or more of its functions develop. Removal of the spleen is therefore indicated in patients with hereditary spherocytosis, autohemolytic anemia and thalassemia because in each case the spleen is associated with specific factors that promote excessive hemolysis. The massive size of the organ in thalassemia is an additional indication for its removal, but in this stage transfusion requirements as a result of the hemolytic mechanism are already excessive. The spleen may be responsible for platelet destruction as in thrombocytopenic purpura. It generates a destructive and/or humoral factor which inhibits the bone marrow not only with regard to platelets but other blood cell precursors as in Banti's and Gaucher's disease resulting in pancytopenia. The increased susceptibility to infection after splenectomy in the young patient emphasizes the need for close supervision for at least two years postoperatively.
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