Disturbed B-lymphocyte selection in autoimmune lymphoproliferative syndrome
نویسندگان
چکیده
منابع مشابه
Disturbed B-lymphocyte selection in autoimmune lymphoproliferative syndrome.
Fas is a transmembrane receptor involved in the maintenance of tolerance and immune homeostasis. In murine models, it has been shown to be essential for deletion of autoreactive B cells in the germinal center. The role of Fas in human B-cell selection and in development of autoimmunity in patients carrying FAS mutations is unclear. We analyzed patients with either a somatic FAS mutation or a ge...
متن کاملDisturbed B-lymphocytes selection in autoimmune lymphoproliferative syndrome
lymphoproliferative syndrome Aleš Janda, Aff1 Aff2 Corresponding Affiliation: Aff1 Klaus Schwarz, Aff3 Aff4 Mirjam van der Burg, Aff5 Werner Vach, Aff6 Hanna Ijspeert, Aff5 Myriam Ricarda Lorenz, Aff3 Magdeldin Elgizouli, Aff1 Kathrin Pieper, Aff1 Paul Fisch, Aff7 Joachim Hagel, Aff1 Raquel Lorenzetti, Aff1 Maximilian Seidl, Aff1 Aff7 Joachim Roesler, Aff8 Fabian Hauck, Aff9 Elisabetta Traggiai...
متن کاملAutoimmune lymphoproliferative syndrome.
PURPOSE OF REVIEW The autoimmune lymphoproliferative syndrome is a recently identified human disorder of lymphocyte apoptosis that has provided important information about Fas-mediated lymphocyte apoptosis. In this review we summarize current information regarding the diagnosis, management and underlying molecular basis of the syndrome. RECENT FINDINGS The genetic basis of autoimmune lymphopr...
متن کاملAutoimmune Lymphoproliferative Syndrome
1»Titu Maiorescu« University, Faculty of Medicine, Department of Physiology, Center for Rheumatic Diseases, Bucharest, Romania 2»Carol Davila« University of Medicine and Pharmacy, Clinic of Neurology, Colentina Clinical Hospital, Bucharest, Romania 3University of Medicine and Pharmacy, Department of Immunology, Craiova, Romania 4»Carol Davila« University of Medicine and Pharmacy, Clinical Hospi...
متن کاملAutoimmune Lymphoproliferative Syndrome Misdiagnosed as Hemophagocytic Lymphohistiocytosis A Case Report
Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consi...
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ژورنال
عنوان ژورنال: Blood
سال: 2016
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2015-04-642488