Jaundice, congenital hemolytic
50 chance to have HS.
The treatment of hereditary spherocytosis is to remove the spleen (splenectomy). Although the red cell defect persists, the breakup of the red cells (hemolysis) ceases. Splenectomy, however, is a hazard in young children. Young children without a spleen are at increased risk for overwhelming sepsis (bloodstream infection), particularly with the pneumococcus bacteria. Splenectomy is therefore usually postponed if possible until the age of 3 years. Before having a splenectomy, anyone with HS should have the pneumococcal vaccine. Persons with HS (or another cause of brisk ongoing hemolysis) should take supplemental folic acid.
The prognosis (outlook) after splenectomy is for a normal life and a normal life expectancy.
HS is also known as severe atypical spherocytosis, spherocytosis type II, ankyrin deficiency, erythrocyte ankyrin deficiency, ankyrin-R deficiency, and ankyrin1 deficiency.
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