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Lymphadenopathy driven by TCR-V γ 8V δ 1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome.

Authors :
Vavassori S
Galson JD
Trück J
van den Berg A
Tamminga RYJ
Magerus-Chatinet A
Pellé O
Camenisch Gross U
Marques Maggio E
Prader S
Opitz L
Nüesch U
Mauracher A
Volkmer B
Speer O
Suda L
Röthlisberger B
Zimmermann DR
Müller R
Diepstra A
Visser L
Haralambieva E
Neven B
Rieux-Laucat F
Pachlopnik Schmid J
Source :
Blood advances [Blood Adv] 2017 Jun 22; Vol. 1 (15), pp. 1101-1106. Date of Electronic Publication: 2017 Jun 22 (Print Publication: 2017).
Publication Year :
2017

Abstract

FAS-dependent apoptosis in V <subscript>δ</subscript> 1 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 V <subscript>δ</subscript> 1 T cell proliferation in ALPS-FAS patients.<br />Competing Interests: Conflict-of-interest disclosure: The authors declare no competing financial interests.

Details

Language :
English
ISSN :
2473-9529
Volume :
1
Issue :
15
Database :
MEDLINE
Journal :
Blood advances
Publication Type :
Academic Journal
Accession number :
29296752
Full Text :
https://doi.org/10.1182/bloodadvances.2017006411