Immunologic Research

, Volume 40, Issue 1, pp 87–92

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

Article

DOI: 10.1007/s12026-007-8001-1

Cite this article as:
Fleisher, T.A. Immunol Res (2008) 40: 87. doi:10.1007/s12026-007-8001-1

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.

Keywords

Apoptosis Autoimmunity Fas Immune homeostasis 

Copyright information

© Humana Press Inc. 2007

Authors and Affiliations

  1. 1.Clinical Center, NIH, DHHSBethesdaUSA

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