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Lymphadenopathy driven by TCR-V γ 8V δ 1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome.
Blood Advances 2017 June 28
FAS-dependent apoptosis in Vδ 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 Vδ 1 T cell proliferation in ALPS-FAS patients.
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