Chediak-Higashi syndrome: clinical, hematologic, and immunologic improvement after splenectomy.
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.
Harfi
H A
HA
Department of Pediatrics and Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
Malik
S A
SA
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