Publication: Hereditary spherocytosis
Date
2004
Authors
Journal Title
Journal ISSN
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Publisher
Kathmandu University
Abstract
Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and
is transmitted as autosomal dominant. Due to this defect there is presence of characteristic spherical cell in
peripheral blood smear and osmotic fragility is increased. Haemolytic anaemia, reticulocytosis, jaundice and
splenomegaly are present. This article reports a case of a 9 year old boy who presented with a history of prolonged
jaundice since the age of 4 years and recurrent pain in the right upper quadrant of abdomen. Clinical examination
revealed jaundice, enlarged liver and marked splenomegaly. Investigations confirmed the diagnosis of hereditary
spherocytosis by the presence of spherocytes in blood smear, raised reticulocytes and increased osmotic fragility.
The patient was subjected to splenectomy after vaccination against coccobacillus and was discharged after proper
advice and on post splenectomy antibiotic prophylaxis.
Key words: Prolonged jaundice, microspherocytes, splenectomy, prophylaxis