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Immunol Res. 2008;40(1):87-92. doi: 10.1007/s12026-007-8001-1.

The autoimmune lymphoproliferative syndrome: an experiment of nature involving lymphocyte apoptosis.

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1
Clinical Center, NIH, DHHS, Bethesda, MD 20892-1508, USA. TFleisher@cc.nih.gov

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.

PMID:
18193364
DOI:
10.1007/s12026-007-8001-1
[Indexed for MEDLINE]
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