SIGNIFICANT AND PERSISTENT IMPROVEMENT OF THROMBOCYTOPENIA AFTER SPLENECTOMY IN AN ADULT WITH THE WISKOTT-ALDRICH SYNDROME AND INTRACEREBRAL BLEEDING

The Wiskott-Aldrich syndrome is an X-linked inherited immunodeficiency disorder characterized by thrombocytopenia, recurrent infections and eczema, Its best management option is HLA-identical bone marrow transplantation; when this is not feasible, splenectomy, followed by continuous prophylactic antibiotics, represents the alternative of choice. The present case report relates the excellent outcome of an adult with the Wiskott-Aldrich syndrome who suffered his first major complication of the disease at age 33 years, an intracerebral hemorrhage, Since an uneventfull splenectomy, thrombocytopenia has significantly improved, and he has remained free of infections for a follow-up period of 3 years while being treated with prophylactic antibiotics. (C) 1995 Wiley-Liss, Inc.