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Splenectomy in Children With “Mild” Hereditary Spherocytosis

Roy, Sani M. MD; Buchanan, George R. MD; Crary, Shelley E. MD, MSCS

Journal of Pediatric Hematology/Oncology: August 2013 - Volume 35 - Issue 6 - p 430–433
doi: 10.1097/MPH.0b013e31829f3460
Original Articles

Splenectomy is considered the treatment of choice for patients with “symptomatic” hereditary spherocytosis (HS). Published guidelines offering recommendations for splenectomy in HS categorize patients primarily based on hemoglobin concentration. We performed a retrospective review of 64 children having splenectomy for HS at Children’s Medical Center Dallas. On the basis of hemoglobin concentration alone, 16 children (25%) had mild, 38 (59%) moderate, and 10 (16%) severe HS. However, when reticulocyte count was used to categorize disease severity, only 3 patients (5%) having splenectomy would be considered mild, 17 (27%) moderate, and 42 (66%) severe. Despite otherwise having “mild” disease defined by near-normal hemoglobin levels, many children with nontraditional or subjective signs and symptoms related to hemolytic rate received a splenectomy, and, therefore, reticulocyte count might be a more reliable laboratory marker suggesting consideration of splenectomy. A validated assessment tool incorporating quality of life indicators in addition to the traditional medical indications for splenectomy in HS would be valuable in assessing indications for the procedure.

*Department of Pediatrics, University of Texas Southwestern Medical Center

Center for Cancer and Blood Disorders, Children’s Medical Center, Dallas, TX

Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR

The authors declare no conflict of interest.

Reprints: Shelley E. Crary, MD, MSCS, Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, 1 Children’s Way, Slot 512-10, Little Rock, AR 72202 (e-mail: secrary@uams.edu).

Received August 1, 2012

Accepted May 7, 2013

Copyright © 2013 Wolters Kluwer Health, Inc. All rights reserved.