Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis

Clinical manifestations of hereditary spherocytosis (HS) can be abrogated b y splenectomy, However, concerns exist regarding exposure of patients to a lifelong risk for overwhelming infections and, to a lesser extent, to vascu lar complications after total splenectomy, In the search for alternative tr eatment modalities, we assessed, in a previous pilot study, the potential u sefulness of subtotal splenectomy in a small population of patients. During a mean follow-up period of 3.5 years, subtotal splenectomy was shown to be effective in decreasing the hemolytic rate, while maintaining the phagocyt ic function of the spleen. In the current study, we evaluated the clinical and biologic features of 40 patients with HS who underwent subtotal splenec tomy and were monitored for periods ranging from 1 to 14 years. The benefic ial effect of subtotal splenectomy included a sustained decrease in hemolyt ic rate and a continued maintenance of phagocytic function of the splenic r emnant. However, mild-to-moderate hemolysis was persistent and accounted fo r secondary gallstone formation and aplastic crisis in a small subset of pa tients. Surprisingly, regrowth of the remnant spleen did not seem to have a major impact on the beneficial outcomes of these individuals. Our results suggest that subtotal splenectomy appears to be a reasonable treatment opti on for management of patients with HS, especially young children. (C) 2001 by The American Society of Hematology.