Natural History of Hereditary Spherocytosis Among Egyptian Pediatric Patients
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- Category: Vol. 79, December 2011
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Natural History of Hereditary Spherocytosis Among Egyptian Pediatric Patients,AMINA ABDELSALAM
Abstract
Objective: This study aimed to better characterize short term and long term natural history of hereditary spherocytosis (HS) in Egyptian pediatric patients.
Design and Methods: A retrospective record review was performed on 40 children with HS. Demographics, age at diagnosis, presenting features, history of complications, hospitalizations and medications were reviewed. Transfusion, neonatal and family history and spleen status were reviewed. All laboratory data at diagnosis and thereafter were revised.
Results: Five (12.5%) cases were mild, 22 (55%) were moderate and 13 (32.5%) of our patients were severe HS. Nine (22.5%) cases presented during the first year of life. Patients presented with intermittent jaundice in 34 (85%), pallor in 36 (90%), dark colored urine in 8 (20%) and abdom-inal distention in 10 (25%). The commonest sign was sple-nomegaly (87.5%), hepatomegaly 18 (45%), cholelithiasis in 3 (7.5%). Blood transfusion at the time of diagnosis was needed in 25 (62.5%) patients. At presentation, the hemoglobin ranged from 3.0-11.0g/dl, reticulocytosis was present in all cases. Spherocytes were seen in 82.5% and Osmotic fragility test (OFT) was positive in 50%. AGLT was performed for 29 cases and was positive in 89.5%. HbF level was seen in 10 cases. Ten (25%) cases were splenectomized and 9/10 required no blood transfusion thereafter and all had no reported com-plications after splenectomy.
Conclusions: Hereditary spherocytosis is not a rare disease in Egypt with its natural history nearly similar to that reported worldwide. However, severe HS was found to be more common among our cases. Gall stones and hypersplenism are the commonest complications. Splenectomy was the most effective and safe line of management of severe cases.