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

Immunologic Research
T A Fleisher

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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Citations

Mar 16, 2013·Proceedings of the National Academy of Sciences of the United States of America·Hong-Ying WangAshish Jain
Oct 21, 2009·Current Opinion in Allergy and Clinical Immunology·Amy P HsuJulie E Niemela
Dec 17, 2009·Hematology·Luigi D Notarangelo
Mar 14, 2014·Endocrine-related Cancer·Shahnawaz ImamJuan Carlos Jaume
Jul 8, 2011·Clinical Rheumatology·László VáróczyArpád Illés
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Jun 7, 2011·Biochemical Pharmacology·Fulvio D'Acquisto, Tessa Crompton
Oct 23, 2012·Journal of Autoimmunity·Owen NgalamikaQianjin Lu
Jan 7, 2014·FEBS Open Bio·Mohammad Mahabub-Uz ZamanShunsuke Ishii
Nov 4, 2008·Current Opinion in Allergy and Clinical Immunology·Joao B OliveiraThomas A Fleisher
Jan 5, 2021·Current Opinion in Rheumatology·Hao Li, George C Tsokos

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