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Blood Adv. 2017 Jun 22;1(15):1101-1106. doi: 10.1182/bloodadvances.2017006411. eCollection 2017 Jun 27.

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

Author information

1
Pediatric Immunology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.
2
Vaccine Group, University of Oxford, Oxford, United Kingdom.
3
Department of Pathology and Medical Biology and.
4
Paediatric Hematology and Oncology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
5
INSERM unité mixte de recherche 1163, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Paris, France.
6
Department of Pathology, University Hospital Zurich, Zurich, Switzerland.
7
Functional Genomics Center Zurich, Swiss Federal Institute of Technology Zurich, University of Zurich, Zurich, Switzerland.
8
Medical Genetics, Institut für Labormedizin, Kantonsspital Aarau, Aarau, Switzerland.
9
Division of Hematology, University Hospital Zurich, Zurich, Switzerland; and.
10
Medical Faculty, University of Zurich, Zurich, Switzerland.

Abstract

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.

Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

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