Hereditary spherocytosis in children: profile and post-splenectomy outcome.

OBJECTIVE

To describe profile of 82 children with hereditary spherocytosis diagnosed over a period of 27 years (1985-2011) from a single center.

METHODS

Retrospective analyses of case records.

RESULTS

The mean (SD) age at diagnosis was 6.7 (2.8) years; 7 (8.5%) were diagnosed in infancy. Pallor (100%), icterus (67%), undocumented fever (28%), splenomegaly (96%) and hepatomegaly (73%) were the most frequent findings. Cholelithiasis was observed in 26%. Twenty-six (32%) underwent splenectomy and were followed for a median duration of 4.5 years. Two (7.7%) children developed post-splenectomy sepsis.

CONCLUSION

Anemia, hepato-splenomegaly and jaundice are commonest clinical features of hereditary spherocytosis. Post-splenectomy sepsis is uncommon.