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

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

•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.

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Veröffentlicht in:Blood advances 2017-06, Vol.1 (15), p.1101-1106
Hauptverfasser: Vavassori, Stefano, Galson, Jacob D., Trück, Johannes, van den Berg, Anke, Tamminga, Rienk Y.J., Magerus-Chatinet, Aude, Pellé, Olivier, Camenisch Gross, Ulrike, Marques Maggio, Ewerton, Prader, Seraina, Opitz, Lennart, Nüesch, Ursina, Mauracher, Andrea, Volkmer, Benjamin, Speer, Oliver, Suda, Luzia, Röthlisberger, Benno, Zimmermann, Dieter Robert, Müller, Rouven, Diepstra, Arjan, Visser, Lydia, Haralambieva, Eugenia, Neven, Bénédicte, Rieux-Laucat, Frédéric, Pachlopnik Schmid, Jana
Format: Artikel
Sprache:eng
Schlagworte:
Exceptional Case Report
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Zusammenfassung:•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.
ISSN:2473-9529
2473-9537
DOI:10.1182/bloodadvances.2017006411