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 and pediatric referral center.

PATIENTS: The presentation, diagnosis, and management of two cases are reported. In addition to the typical features of WAS, the first patient had hemolytic anemia, arthritis, leukocytoclastic vasculitis, and colitis. The second patient had colitis and 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 transplantation is the only successful mode of treatment for all aspects of WAS.