Anirban Das1, Deepak Bansal, Reena Das, Amita Trehan, R K Marwaha. 1. Pediatric Hematology-oncology unit, Advanced Pediatric Center and *Department of Hematology, PGIMER, Chandigarh, India. Correspondence to: Dr Deepak Bansal, Hematology-oncology unit, Department of Pediatrics, Advanced Pediatric Center, PGIMER, Chandigarh, India. deepakbansaldr@gmail.com.
Abstract
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.
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.