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Blood, 26 March 2009, Vol. 113, No. 13, pp. 3027-3030. Prepublished online as a Blood First Edition Paper on January 27, 2009; DOI 10.1182/blood-2008-09-179630. This Article Full Text (PDF) Supplemental Figure All Versions of this Article: blood-2008-09-179630v1 113/13/3027    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Magerus-Chatinet, A. Articles by Rieux-Laucat, F. Search for Related Content PubMed PubMed Citation Articles by Magerus-Chatinet, A. Articles by Rieux-Laucat, F. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted September 18, 2008 Accepted December 21, 2008 FAS-L, IL-10, and double-negative CD4-CD8-TCR alpha/beta+ T cells are reliable markers of ALPS associated with FAS loss of function Aude Magerus-Chatinet, Marie-Claude Stolzenberg, Maria S. Loffredo, Benedicte Neven, Catherine Schaffner, Nicolas Ducrot, Peter D. Arkwright, Brigitte Bader-Meunier, Jose Barbot, Stephane Blanche, Jean-Laurent Casanova, Marianne Debre, Alina Ferster, Claire Fieschi, Benoit Florkin, Claire Galambrun, Olivier Hermine, Olivier Lambotte, Eric Solary, Caroline Thomas, Francoise Le Deist, Capucine Picard, Alain Fischer, and Frederic Rieux-Laucat* Inserm, U768, Paris, France Universite Paris Descartes, Paris, France Department of Pediatrics, School of Medicine, University of Bari, Bari, Italy Hopital Necker-Enfants Malades, Paris, France Department of Pediatric Immunology, Newcastle General Hospital, Newcastle upon Tyne, United Kingdom Unite d'Immunologie et d'Hematologie Pediatrique, Paris, France Service d'Hematologie, Hopital de Criancas Maria Pia, Porto, Portugal Assistance Publique des Hopitaux de Paris, Paris, France Inserm U550, Paris, France Hopital Universitaire des Enfants-Reine Fabiola, Bruxelles, Belgium Unite d'immunopathologie, Departement d'Immunologie Clinique, Hopital Saint-Louis, Paris, France Departement de Pediatrie, Centre Hospitalier Universitaire de Liege, Liege, Belgium Service d'hematologie pediatrique, Hopital des enfants de la Timone, Marseille, France CNRS UMR 8147, Service d'hematologie et centre de reference des mastocytoses, Paris, France Service de Medecine Interne, Hopital de Bicetre, Le Kremlin-Bicetre, France UMR 866, Faculte de Medecine, Dijon, France Service d'Oncologie Pediatrique, Hopital Mere Enfant, Nantes, France Department of Microbiology and Immunology, CHU Sainte-Justine, University of Montreal, Montreal, Canada Centre d'Etude des Deficits Immunitaires, Paris, France * Corresponding author; email: frederic.rieux-laucat{at}inserm.fr. Autoimmune lymphoproliferative syndrome (ALPS) is characterized by splenomegaly, lymphadenopathy, hypergammaglobulinemia, accumulation of double-negative TCR+CD4-CD8- T cells (DNT cells) and autoimmunity. Previously, DNT cells detection and a functional defect of T cells in a FAS-induced apoptosis test in vitro have been used for ALPS diagnosis. However, a functional defect can also be detected in mutation-positive relatives (MPRs) who remain free of any ALPS-related disease. In contrast, lymphocytes from patients carrying a somatic mutation of FAS exhibit normal sensitivity to FAS-induced apoptosis in vitro. We assessed the soluble FAS-L concentration in the plasma of ALPS patients carrying FAS mutations. Overall, we showed that determination of the FAS-L represents, together with the IL-10 concentration and the DNT cells percentage, a reliable tool for the diagnosis of ALPS. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Michel, V. Chanet, A. Dechartres, A.-S. Morin, J.-C. Piette, L. Cirasino, G. Emilia, F. Zaja, M. Ruggeri, E. Andres, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases Blood, October 8, 2009; 114(15): 3167 - 3172. [Abstract] [Full Text] [PDF]

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