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Lymphadenopathy driven by TCR-Vγ8Vδ1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome
Stefano Vavassori1, Jacob D Galson1,2, Johannes Trück1
1Pediatric Immunology and Children's Research Center, University Children's Hospital 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.
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