Article
The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions.
Journal of clinical immunology - 1 Jul 2018
Rieux-Laucat Frédéric, Magérus-Chatinet Aude, Neven Bénédicte
Abstract excerpt
The autoimmune lymphoproliferative syndrome (ALPS) is a non-malignant and non-infectious uncontrolled proliferation of lymphocytes accompanied by autoimmune cytopenia. The genetic etiology of the ALPS was described in 1995 by the discovery of the FAS gene mutations. The related apoptosis defect accounts for the accumulation of autoreactive lymphocytes as well as for specific clinical and biological features that...
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