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American Society of Hematology, Blood Advances, 15(1), p. 1101-1106, 2017

DOI: 10.1182/bloodadvances.2017006411

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Lymphadenopathy driven by TCR-Vγ8Vδ1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome

Journal article published in 2017 by Stefano Vavassori, Jacob D. Galson, Johannes Trück, Anke van den Berg, Rienk Y. J. Tamminga, Aude Magerus-Chatinet, Olivier Pellé, Ulrike Camenisch Gross, Ewerton Marques Maggio, Seraina Prader, Lennart Opitz, Ursina Nüesch, Andrea Mauracher, Benjamin Volkmer, Oliver Speer and other authors.
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

Key Points FAS-dependent apoptosis in Vδ1 T cells makes the latter possible culprits for the lymphadenopathy observed in patients with FAS mutations. Rapamycin and methylprednisolone resistance should prompt clinicians to look for Vδ1 T cell proliferation in ALPS-FAS patients.