AUTOIMMUNE MANIFESTATIONS OF THE WISKOTT-ALDRICH-SYNDROME

Objective: To describe and review the autoimmune features and typical manifestations of Wiskott-Aldrich syndrome (WAS). Design: Case series and review of the literature. Setting: Tertiary care medical center an d pediatric referral center, Patients: The presentation, diagnosis, an d management of two cases are reported. In addition to the typical fea tures of WAS, the first patient had hemolytic anemia, arthritis, leuko cytoclastic vasculitis, and colitis, The second patient had colitis an d arthralgias. Detailed review of features and therapeutic options in WAS as exemplified by these two patients are presented, Both patients had bone marrow transplantation, the only definitive treatment for WAS . Conclusions: WAS has variable clinical and autoimmune manifestations . Diagnosis must be suspected in a boy with small, decreased number of platelets and autoimmune problems or infections, Bone marrow transpla ntation is the only successful mode of treatment for all aspects of WA S, Copyright (C) 1998 by W.B. Saunders Company.