Microesferocitosis Familiar Estudio en 10 Familias

A 6 1/2 years old girl admitted to our hospital with an aplastic crisis, a serious complication of HereditarySpherocytosis (H.S.), motivated a retrospective review of the cases studies in our Hematology Department duringthe last seven years. Ten families were studied finding 20 members with H.S. The diagnosis was made in most ofthem during school age and only in two patients during the neonatal period. Most patients (80%t had objectivefamily history of H.S. Hemolytic crisis represented 80%, aplastic crisis 15% and cholelithiasis 5 % of thecomplications detected among our patients. The relationships that exist between aplastic crisis and viral Infections,specially parvo-virus, is stressed. It is important to point out that two out of three mothers who were not treatedby splenectomy, showed severe hemolitic crisis during their pregnancy. Splenectomy, its indications and posterioranti-infective prophylaxis are discussed.(Keywords: Anemia. Hemolysis. Hereditary Spherocytosis. Aplastic crisis. Splenectomy).