Study of Hereditary Spherocytosis (HS) at a Single Institute

Clinical Course of the Patients and Outstanding Issues about Splenectomy

Abstract

We investigated the clinical aspects of nine families and 12 children with hereditary spherocytosis, including diagnosis, complication, justification of splenectomy, and clinical course after splenectomy. Median age at diagnosis was 5.5 years (1 month to 11 years). Seven cases (58%) had a family history. In the neonatal period, 10 patients received phototherapy and 1 patient had exchange transfusion due to neonatal hyperbilirubinemia. Laboratory findings at diagnosis were : Hb of 7.4 2.1 g/dl, reticulocyte count of (27.6±22.1) ×10⁴/μl, and total bilirubin of 2.2±0.9 mg/dl. Seven cases (58%) concurred aplastic crisis due to human parvovirus B19 infection. Six patients received erythrocyte transfusions. Of the seven patients who underwent splenectomy, 6 were more than 5 years of age. All patients recovered Hb and had elevated platelet counts after splenectomy. All patients received pneumococcal vaccination (Pneumovax^®) before splenectomy and subsequent postsplenectomy infection was not observed. Splenectomy, on one hand, contributes not only to the recovery of anemia, but also to prevention of gallstone and hemolytic crisis. On the other hand, there is a small risk of subsequent postsplenectomy infection and ischemic heart disease. So it is requisite for practitioners to follow up their patients carefully.