Splenectomy in children with mild hereditary spherocytosis

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.