Lymphadenopathy driven by TCR-V?8V?1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome.
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ABSTRACT:
SUBMITTER: Vavassori S
PROVIDER: S-EPMC5728321 | biostudies-literature | 2017 Jun
REPOSITORIES: biostudies-literature
Publications
Lymphadenopathy driven by TCR-V<sub>γ</sub>8V<sub>δ</sub>1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome.
Vavassori Stefano S Galson Jacob D JD Trück Johannes J van den Berg Anke A Tamminga Rienk Y J RYJ Magerus-Chatinet Aude A Pellé Olivier O Camenisch Gross Ulrike U Marques Maggio Ewerton E Prader Seraina S Opitz Lennart L Nüesch Ursina U Mauracher Andrea A Volkmer Benjamin B Speer Oliver O Suda Luzia L Röthlisberger Benno B Zimmermann Dieter Robert DR Müller Rouven R Diepstra Arjan A Visser Lydia L Haralambieva Eugenia E Neven Bénédicte B Rieux-Laucat Frédéric F Pachlopnik Schmid Jana J
Blood advances 20170622 15
FAS-dependent apoptosis in V<sub>δ</sub>1 T cells makes the latter possible culprits for the lymphadenopathy observed in patients with <i>FAS</i> mutations.Rapamycin and methylprednisolone resistance should prompt clinicians to look for V<sub>δ</sub>1 T cell proliferation in ALPS-FAS patients. ...[more]
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