SH Ang, JCM Lam

Abstract

Purpose: Hereditary spherocytosis (HS) is a red cell membrane disorder less commonly seen in Asian populations. This study highlights the diagnostic features and complications of HS patients followed up in an Asian paediatric institution. Methods: Data was obtained from retrospective medical records of paediatric patients diagnosed with HS from 2006-2016. Findings: Thirty-one patients were identified with a mean age (MA) at diagnosis of 3.5 years (0-14.3). Sixteen patients (52%) had positive family history. Twelve patients (38.7%) presented early (<1 month), most with neonatal jaundice. Twenty-one patients (67.7%) required at least one transfusion. Gallstones were detected in 10 patients (32.3%) - 4 required cholecystectomy (MA 11.7 years). Six patients (19.4%) had splenectomy (MA 12.5 years), mostly due to frequent transfusions. Conclusions: Most paediatric patients in our institution are diagnosed early, with the majority requiring at least one transfusion. HS should be considered in neonates presenting with early jaundice requiring phototherapy.