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  • Title: The autoimmune lymphoproliferative syndrome: an experiment of nature involving lymphocyte apoptosis.
    Author: Fleisher TA.
    Journal: Immunol Res; 2008; 40(1):87-92. PubMed ID: 18193364.
    Abstract:
    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.
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