Immune Thrombocytopenia Treated with High-Dose Intravenous Gamma Globulin and Corticosteroids in Two Patients with Wiskott-Aldrich Syndrome

Abstract

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disorder characterized by thrombocytopenia, atopic dermatitis, and susceptibility to recurrent infections. The thrombocytopenia, due to an intrinsic platelet defect, is only rarely complicated by immune thrombocytopenia (ITP). We report two patients with WAS in whom ITP complicated their thrombocytopenia. In addition, immune neutropenia accompanied the ITP in one patient. Whereas administration of high-dose intravenous gamma globulin (IGIV) alone did not ameliorate the ITP, corticosteroids did improve the ITP and immune neutropenia. High-dose IGIV appeared to be synergistic with corticosteroids and thus have a steroid-sparing effect.