Lymphadenopathy driven by TCR-Vγ8Vδ1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome
نویسندگان
چکیده
منابع مشابه
Autoimmune lymphoproliferative syndrome with somatic Fas mutations.
BACKGROUND Impaired Fas-induced apoptosis of lymphocytes in vitro is a principal feature of the autoimmune lymphoproliferative syndrome (ALPS). We studied six children with ALPS whose lymphocytes had normal sensitivity to Fas-induced apoptosis in vitro. METHODS Susceptibility to Fas-mediated apoptosis and the Fas gene were analyzed in purified subgroups of T cells and other mononuclear cells ...
متن کاملAutoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy.
Autoimmune lymphoproliferative syndrome (ALPS) is an uncommon nonmalignant lymphoproliferative disease which is characterized by chronic, persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia , hypergammaglobinemia and increased risk of lymphoma. We report a 2-year old boy with hepatosplenomegaly as first presentation. Petechial and purpuric rashes with massive c...
متن کاملautoimmune lymphoproliferative syndrome (alps) in a boy with massive lymphadenopathy
autoimmune lymphoproliferative syndrome (alps) is an uncommon nonmalignant lymphoproliferative disease which is characterized by chronic, persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia, hypergammaglobinemia and increased risk of lymphoma. we report a 2-year old boy with hepatosplenomegaly as first presentation. petechial and purpuric rashes with massive ce...
متن کاملFAS-L, IL-10, and double-negative CD4 CD8 TCR / T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function
Aude Magerus-Chatinet,1,2 Marie-Claude Stolzenberg,1,2 Maria S. Loffredo,1,2 Bénédicte Neven,1-3 Catherine Schaffner,1,2 Nicolas Ducrot,1,2 Peter D. Arkwright,4 Brigitte Bader-Meunier,3 José Barbot,5 Stéphane Blanche,3 Jean-Laurent Casanova,2,3,6 Marianne Debré,3 Alina Ferster,7 Claire Fieschi,8 Benoit Florkin,9 Claire Galambrun,10 Olivier Hermine,2-3,11 Olivier Lambotte,12 Eric Solary,13 Carol...
متن کاملDiagnosis of autoimmune lymphoproliferative syndrome caused by FAS deficiency in adults.
A diagnosis of autoimmune lymphoproliferative syndrome caused by FAS deficiency during adulthood is unusual. We analyzed 17 cases of autoimmune lymphoproliferative syndrome caused by FAS deficiency diagnosed during adulthood in French reference centers for hereditary immunodeficiencies and for immune cytopenias. Twelve of the 17 patients had developed their first symptoms during childhood. The ...
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ژورنال
عنوان ژورنال: Blood Advances
سال: 2017
ISSN: 2473-9529,2473-9537
DOI: 10.1182/bloodadvances.2017006411