Autoimmune lymphoproliferative syndrome - A human disorder of abnormal lymphocyte survival

被引：64

作者：

Bleesing, JJH

Straus, SE

Fleisher, TA

机构：

[1] NIAID, Dept Lab Med, Warren G Magnuson Clin Ctr, NIH, Bethesda, MD 20892 USA

[2] NIAID, Clin Invest Lab, NIH, Bethesda, MD 20892 USA

[3] Uniformed Serv Univ Hlth Sci, Dept Pediat, Bethesda, MD 20814 USA

来源：

PEDIATRIC CLINICS OF NORTH AMERICA | 2000年 / 47卷 / 06期

关键词：

D O I：

10.1016/S0031-3955(05)70272-8

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

An important aspect of the immune system is the maintenance of a homeostatic balance between lymphocyte survival and death. This is regulated by apoptosis (programmed cell death). One pathway of this active process of cell suicide is mediated by the lymphocyte receptor Fas (CD95). Its importance in this homeostatic balance is underscored by the consequences of defective Pas-mediated apoptosis in patients with autoimmune lymphoproliferative syndrome (ALPS). Abnormal accumulation of lymphocytes results in lymphadenopathy, hepatosplenomegaly, and hypersplenism Failure of removal of potentially autoreactive lymphocytes is associated with the appearance of autoimmune manifestations. Finally, inappropriate survival of lymphocytes may lead to the development of malignancies.

引用

页码：1291 / +

页数：21

共 67 条

[1] Identification of new Fas mutations in a patient with autoimmune lymphoproliferative syndrome (ALPS) and eosinophilia [J].