title: Chediak-Higashi syndrome: clinical, hematologic, and immunologic improvement after splenectomy.
abstract: A 10-year-old boy with Chediak-Higashi syndrome in accelerated phase failed to respond to treatment with ascorbic acid, vincristine, and prednisone. Splenectomy resulted in clinical, hematologic, and immunologic improvement: his leukocyte chemotactic and phagocytic functions returned to normal. We suggest that splenectomy be considered in treatment of the accelerated phase of Chediak-Higashi syndrome unresponsive to other forms of therapy.
authors.affiliation: Department of Pediatrics and Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
authors.forename: H A
authors.initials: HA
authors.lastname: Harfi
authors.forename: S A
authors.initials: SA
authors.lastname: Malik
mesh_terms: D000917:Antibody Formation; D002609:Chediak-Higashi Syndrome / Q000276:immunology / Q000601:surgery / Q000628:therapy*; D002648:Child; D006801:Humans; D007111:Immunity, Cellular; D008297:Male; D009504:Neutrophils / Q000502:physiology; D013156:Splenectomy