Partial splenectomy for hereditary spherocytosis: a multi-institutional review |
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Authors: | Buesing Keely L Tracy Elisabeth T Kiernan Colleen Pastor Aimee C Cassidy Laura D Scott J Paul Ware Russell E Davidoff Andrew M Rescorla Frederick J Langer Jacob C Rice Henry E Oldham Keith T |
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Institution: | a Children's Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, WI 53226, USAb Duke University Medical Center, Durham, NC, USAc Riley Hospital for Children/Indiana University School of Medicine, Indianapolis, IN, USAd The Hospital for Sick Children/University of Toronto, Toronto, ON, Canadae St Jude Children's Research Hospital, Memphis, TN, USA |
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Abstract: | Background/PurposePartial splenectomy has emerged as a surgical option for selected children with hereditary spherocytosis, with the goal of reducing anemia while preserving splenic function. This multi-institutional study is the largest series to date examining outcomes data for partial splenectomy in patients with hereditary spherocytosis.MethodsData were collected retrospectively from 5 North American pediatric hospitals. Sixty-two children underwent partial splenectomy for hereditary spherocytosis between 1990 and 2008.ResultsAt 1 year following partial splenectomy, mean hemoglobin significantly increased by 3.0 ± 1.4 g/dL (n = 52), reticulocyte count decreased by 6.6% ± 6.6% (n = 41), and bilirubin level decreased by 1.3 ± 0.9 mg/dL (n = 25). Patients with poor or transient hematologic response were found to have significantly more splenic regeneration postoperatively compared with patients with a durable clinical response (maximal spleen dimension, 9.0 ± 3.4 vs 6.3 ± 2.2 cm). Clinically significant recurrence of anemia or abdominal pain led to completion splenectomy in 4.84% of patients. No patients developed postsplenectomy sepsis.ConclusionsOur multi-institutional review indicates that partial splenectomy for hereditary spherocytosis leads to sustained and clinically significant improvement in hematologic profiles and clinical symptoms in most patients. Our data support partial splenectomy as an alternative for selected children with hereditary spherocytosis. |
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Keywords: | Hereditary spherocytosis Partial splenectomy Multi-institutional review Outcomes data |
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