FAS相关自身免疫性淋巴增殖综合征中由TCR-V8V1 T细胞扩增驱动的淋巴结病
Lymphadenopathy driven by TCR-V8V1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome.
作者信息
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
机构信息
Pediatric Immunology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.
Vaccine Group, University of Oxford, Oxford, United Kingdom.
出版信息
Blood Adv. 2017 Jun 22;1(15):1101-1106. doi: 10.1182/bloodadvances.2017006411. eCollection 2017 Jun 27.
FAS-dependent apoptosis in V1 T cells makes the latter possible culprits for the lymphadenopathy observed in patients with mutations.Rapamycin and methylprednisolone resistance should prompt clinicians to look for V1 T cell proliferation in ALPS-FAS patients.
V1 T细胞中FAS依赖的凋亡使得后者可能是突变患者中观察到的淋巴结病的罪魁祸首。雷帕霉素和甲基强的松龙耐药应促使临床医生在ALPS-FAS患者中寻找V1 T细胞增殖情况。
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