FAS-L, IL-10, and double-negative CD4- CD8- TCR alpha/beta+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function.

doi:10.1182/blood-2008-09-179630

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Article

FAS-L, IL-10, and double-negative CD4- CD8- TCR alpha/beta+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function.

Blood - 26 Mar 2009

Magerus-Chatinet Aude, Stolzenberg Marie-Claude, Loffredo Maria S, Neven Bénédicte, Schaffner Catherine, Ducrot Nicolas, Arkwright Peter D, Bader-Meunier Brigitte, Barbot José, Blanche Stéphane, Casanova Jean-Laurent, Debré Marianne, Ferster Alina, Fieschi Claire, Florkin Benoit, Galambrun Claire, Hermine Olivier, Lambotte Olivier, Solary Eric, Thomas Caroline, Le Deist Francoise, Picard Capucine, Fischer Alain, Rieux-Laucat Frédéric

Abstract excerpt

Autoimmune lymphoproliferative syndrome (ALPS) is characterized by splenomegaly, lymphadenopathy, hypergammaglobulinemia, accumulation of double-negative TCRalphabeta(+) CD4(-)CD8(-) T cells (DNT cells), and autoimmunity. Previously, DNT cell detection and a functional defect of T cells in a FAS-induced apoptosis test in vitro had been used for ALPS diagnosis. However, a functional defect can also be detected in...

Topics

Adolescent
Adult
Autoimmune Diseases
Biomarkers
CD4 Antigens
CD8 Antigens
Case-Control Studies
Child

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Child, Preschool

Fas Ligand Protein

Humans

Infant

Infant, Newborn

Interleukin-10

Lymphoproliferative Disorders

Middle Aged

Mutation

Receptors, Antigen, T-Cell, alpha-beta

Source

Open in PubMed

Identifiers

PMID: 19176318
DOI: 10.1182/blood-2008-09-179630

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