期刊
PEDIATRIC BLOOD & CANCER
卷 53, 期 6, 页码 1117-1119出版社
WILEY-LISS
DOI: 10.1002/pbc.22151
关键词
immune cytopenias; immunology; pharmacology
Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the immune System Caused by inadequate induction of apoptosis via the Fas pathway, mainly characterized by generalized lymphadenopathy, splenomegaly, and autoimmune cytopenias, as well as increased risk of lymphoma. Although the clinical Course of ALPS is highly variable, without treatment long-term prognosis is unsatisfactory for most patients. ALPS has been treated with most of the existing immunosuppressive agents, with variable success. We hereby present a case of a child with ALPS whose greatly enlarged lymph nodes rapidly regressed upon initiation of rapamycin, a novel potential therapeutic agent in the treatment of ALPS. Pediatr Blood Cancer 2009;53:1117-1119. (C) 2009 Wiley-Liss, Inc.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据