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Hereditary spherocytosis in children: profile and post-splenectomy outcome
Anirban Das1, Deepak Bansal, Reena Das
1Pediatric 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.
Insights
Hereditary spherocytosis in children presents with anemia, enlarged spleen and liver, and jaundice. Post-splenectomy sepsis is rare in these patients.
Area of Science:
- Hematology
- Pediatric Medicine
- Genetics
Background:
- Hereditary spherocytosis (HS) is a genetic hemolytic anemia.
- It is characterized by abnormal red blood cell shape and splenic sequestration.
- HS presents with variable clinical severity in children.
Purpose of the Study:
- To characterize the clinical profile of pediatric hereditary spherocytosis.
- To analyze diagnostic features and management outcomes over 27 years.
Main Methods:
- Retrospective analysis of case records.
- Involved 82 children diagnosed with hereditary spherocytosis between 1985 and 2011.
Main Results:
- Mean age at diagnosis was 6.7 years; 8.5% diagnosed in infancy.
- Common findings included pallor (100%), splenomegaly (96%), hepatomegaly (73%), and jaundice (67%).
- Gallstones occurred in 26%; 32% underwent splenectomy with 7.7% developing post-splenectomy sepsis.
Conclusions:
- Anemia, hepato-splenomegaly, and jaundice are key clinical features of pediatric HS.
- Splenectomy is a treatment option, and post-splenectomy sepsis is infrequent.
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.
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