title: 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.
authors.affiliation: Département de Pédiatrie, Laboratoire d'Hématologie, Service de médecine nucléaire, Service d'épidémiologie et de statistique, INSERM U 292, Hôpital Bicétre, Le Kremlin Bicétre, France.
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: F
authors.initials: F
authors.lastname: Gauthier
authors.forename: F
authors.initials: F
authors.lastname: Archambaud
authors.forename: T
authors.initials: T
authors.lastname: Cynober
authors.forename: F
authors.initials: F
authors.lastname: Miélot
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
authors.forename: J
authors.initials: J
authors.lastname: Warszawski
authors.forename: N
authors.initials: N
authors.lastname: Mohandas
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
mesh_terms: D000293:Adolescent; D000328:Adult; D001827:Body Height; D002648:Child; D002675:Child, Preschool; D002769:Cholelithiasis / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000378:metabolism; D006461:Hemolysis; D006801:Humans; D007223:Infant; D008297:Male; D010587:Phagocytosis / Q000502:physiology; D010976:Platelet Count; D011788:Quality of Life; D017701:Reticulocyte Count; D013103:Spherocytosis, Hereditary / Q000150:complications / Q000601:surgery*; D013154:Spleen / Q000254:growth & development; D013156:Splenectomy / Q000009:adverse effects / Q000379:methods / Q000592:standards
chemical_list: D006454:Hemoglobins