title: Effectiveness of partial splenectomy in hereditary spherocytosis.
abstract: Total splenectomy eliminates the splenic destruction of erythrocytes with impaired deformability. However, concern has increased over the lifelong risk of overwhelming postsplenectomy infections in splenectomized patients, a risk reduced but not totally suppressed by appropriate prophylaxis. Partial splenectomy, as long as 80% to 90% of the enlarged spleen is removed and less than 25% of the normal spleen volume is retained, is a logical alternative, both preserving the phagocytic and immune function of the spleen and decreasing erythrocyte destruction. A 12-year experience has shown that subtotal splenectomy is efficient in decreasing hemolysis, although to a lesser extent than total splenectomy, with sustained results over years in most patients, and indirect evidence argues for the integrity of phagocytic function. Such a surgical procedure should be considered in transfusion-dependent infants with hereditary spherocytosis and in older patients with erythrocyte membrane defects, provided further follow-up confirms the experience of the past 12 years.
authors.affiliation: Department of Hematology, Centre Hospitalier de Bicêtre, Le Kremlin Bicêtre, France.
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: P
authors.initials: P
authors.lastname: Berterottiere
authors.forename: S
authors.initials: S
authors.lastname: Eber
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
authors.forename: F
authors.initials: F
authors.lastname: Gauthier
mesh_terms: D006801:Humans; D013103:Spherocytosis, Hereditary / Q000601:surgery*; D013156:Splenectomy