Autoimmune manifestations of the Wiskott-Aldrich syndrome

被引:25
作者
Akman, IO
Ostrov, BE
Neudorf, S
机构
[1] Penn State Univ, Milton S Hershey Med Ctr, Dept Pediat, Hershey, PA 17033 USA
[2] Childrens Hosp Pittsburgh, Pittsburgh, PA 15213 USA
关键词
Wiskott-Aldrich syndrome; autoimmune disease; arthritis; colitis;
D O I
10.1016/S0049-0172(98)80002-4
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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, Copyright (C) 1998 by W.B. Saunders Company.
引用
收藏
页码:218 / 225
页数:8
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