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

Abstract

Clinical manifestations of hereditary spherocytosis (HS) can be abrogated by splenectomy. However, concerns exist regarding exposure of patients to a lifelong risk for overwhelming infections and, to a lesser extent, to vascular complications after total splenectomy. In the search for alternative treatment modalities, we assessed, in a previous pilot study, the potential usefulness 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 phagocytic function of the spleen. In the current study, we evaluated the clinical and biologic features of 40 patients with HS who underwent subtotal splenectomy and were monitored for periods ranging from 1 to 14 years. The beneficial effect of subtotal splenectomy included a sustained decrease in hemolytic rate and a continued maintenance of phagocytic function of the splenic remnant. However, mild-to-moderate hemolysis was persistent and accounted for secondary gallstone formation and aplastic crisis in a small subset of patients. 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 option for management of patients with HS, especially young children.

Hereditary Spherocytosis - Diagnosis, Surgical Treatment and Outcomes. A Literature Review

“…In the case of a partial splenectomy, an important concern is the regrowth of the remnant, consistently noted in many previous reports (25,33,35). This disadvantage appears to be more frequently encountered in young children.…”

“…(22)(23)(24) Several studies have set the minimal amount of the residual parenchyma necessary to maintain adequate phagocytic function, to approximately 15% of the enlarged spleen. (25)(26)(27) Technically, a splenectomy can be performed either in the traditional open manner or with a minimally invasive approach.…”

Esferocitosis hereditaria. Revisión. ParteII. Manifestaciones clínicas, evolución, complicaciones y tratamiento

“…67 Sin embargo, Bader-Meunier et al comunicaron que 3 de una serie de 40 casos debieron ser sometidos luego a esplenectomía total. 68 En el estudio más grande publicado, sobre 62 niños, el porcentaje de pacientes que requirió posteriormente esplenectomía total fue menor del 5%. 69 La esplenectomía "casi total", en la cual se deja un fragmento de tejido esplénico de 10 cm 3 , está aún siendo evaluada.…”

Red Cell Membrane Disorders

“…Recently, partial splenectomy via laparotomy has been advocated for infants and young children with severe, usually transfusion-dependent, anemia. 11,12 The goal is to palliate the hemolysis and anemia while maintaining residual splenic immune function. Long-term data for this procedure are lacking, but rapid regrowth of the spleen may limit its effectiveness in some cases.…”