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: H A Harfi, S A Malik
• Journal: Annals of allergy (Ann Allergy) Vol. 69 Issue 2 Pg. 147-50 (Aug 1992) ISSN: 0003-4738 [Print] United States
• PMID: 1510287 (Publication Type: Case Reports, Journal Article)

Topics

• Antibody Formation
• Chediak-Higashi Syndrome (immunology, surgery, therapy)
• Child
• Humans
• Immunity, Cellular
• Male
• Neutrophils (physiology)
• Splenectomy