Autoimmune lymphoproliferative syndrome - A genetic disorder of abnormal lymphocyte apoptosis

被引：13

作者：

Bleesing, JJH ^{[1
]}

机构：

[1] Univ Arkansas Med Sci, Arkansas Childrens Hosp, Inst Res, Dept Pediat, Little Rock, AR 72202 USA

来源：

IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA | 2002年 / 22卷 / 02期

关键词：

D O I：

10.1016/S0889-8561(01)00011-X

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

Fas-mediated apoptosis has an important role in the maintenance of a homeostatic balance between lymphocyte survival and death. This role is underscored by three consequences of a genetic disorder of abnormal Fas-mediated apoptosis, referred to as autoimmune lymphoproliferative syndrome. Abnormal proliferation of lymphocytes increases the risk for malignancies and results in lymphadenopathy, hepatosplenomegaly and hypersplenism, failure to remove autoreactive lymphocytes (associated with autoimmunity), and inappropriate survival of lymphocytes.

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页码：339 / +

页数：18

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