Autoimmune lymphproliferative syndrome (ALPS) is a human disorder that has been characterized in the past two decades at both a functional and a genetic level. The underlying basis for this disorder is a defect in lymphocyte apoptosis that alters immune homeostasis resulting in an expansion of a normally rare circulating lymphocyte, the alpha beta double negative T cell. The abnormality in Fas mediated apoptosis underlying ALPS serves as a risk factor for autoimmunity involving blood cells and the development of lymphoma. There remain patients with a diagnosis of ALPS but without a defined genetic defect and current investigations are focusing on fully characterizing this patient subgroup. © 2007 Humana Press Inc.
CITATION STYLE
Fleisher, T. A. (2008). The autoimmune lymphoproliferative syndrome: An experiment of nature involving lymphocyte apoptosis. Immunologic Research, 40(1), 87–92. https://doi.org/10.1007/s12026-007-8001-1
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