HEREDITARY SPHEROCYTOSIS IN A MALAY PATIENT WITH CHRONIC HAEMOLYSIS

This case report describes a 35-year-old lady who presented with generalizedweakness and lethargy of two weeks duration and jaundice of more than 20 yearsduration. Her initial workup was suggestive of haemolysis and blood film showeda leucoerythoblastic picture with moderate microspherocytes. She was finallydiagnosed as a case of hereditary spherocytosis after ruling out other possible causesof chronic haemolysis and supported by an abnormal osmotic fragility test, althoughfamily members refused for screening. Hereditory spherocytosis is uncommon inMalay population and presentation with jaundice of 20 years duration withleucoerythroblastic picture on blood film were interesting features in this case.Patient is being followed closely for need of splenectomy in near future as perseverity of haemolysis and currently being managed with folic acid supplement