Hereditary spherocytosis in a malay patient with chronic haemolysis.

Malays J Med Sci

Advanced Medical and Dental Institute (Clinical Centre, Universiti Sains Malaysia, No. 29 Lorong Bertam Indah 4/9, Taman Bertam Indah, 13200 Kepala Batas, Penang, Malaysia.

Published: July 2007

This case report describes a 35-year-old lady who presented with generalized weakness and lethargy of two weeks duration and jaundice of more than 20 years duration. Her initial workup was suggestive of haemolysis and blood film showed a leucoerythoblastic picture with moderate microspherocytes. She was finally diagnosed as a case of hereditary spherocytosis after ruling out other possible causes of chronic haemolysis and supported by an abnormal osmotic fragility test, although family members refused for screening. Hereditory spherocytosis is uncommon in Malay population and presentation with jaundice of 20 years duration with leucoerythroblastic picture on blood film were interesting features in this case. Patient is being followed closely for need of splenectomy in near future as per severity of haemolysis and currently being managed with folic acid supplement.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3442627PMC

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