Case report: Effectiveness of sirolimus in a de novo FAS mutation leading to autoimmune lymphoproliferative syndrome-FAS and elevated DNT/Treg ratio

Case report: Effectiveness of sirolimus in a de novo FAS mutation leading to autoimmune lymphoproliferative syndrome-FAS and elevated DNT/Treg ratio

BackgroundThe autoimmune lymphoproliferative syndrome (ALPS) is a rare disease characterized by defective function of the FAS death receptor, which results in chronic, non-malignant lymphoproliferation and autoimmunity accompanied by elevated numbers of double-negative (DN) T cells (T-cell receptor...

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Bibliographic Details
Main Authors: Hao Gu, Zhenping Chen, Jie Ma, Jingyao Ma, Lingling Fu, Rui Zhang, Tianyou Wang, Runhui Wu
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-07-01
Series:Frontiers in Pediatrics
Subjects:
FAS (APO-1/CD95)
ALPS (autoimmune lymphoproliferative syndrome)
sirolimus (rapamycin)
DNT
Treg - regulatory T-cell
Online Access:https://www.frontiersin.org/articles/10.3389/fped.2022.868193/full

Internet

https://www.frontiersin.org/articles/10.3389/fped.2022.868193/full

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