Immunophenotyping of A20 haploinsufficiency by multicolor flow cytometry
Immunophenotyping of A20 haploinsufficiency by multicolor flow cytometry
复制标题
DOI：
10.1016/j.clim.2020.108441
复制
发表时间：
2020-07-01
影响因子：
8.6
通讯作者：
Fukao, Toshiyuki
中科院分区：
文献类型：
--
作者：
Kadowaki, Tomonori;Ohnishi, Hidenori;Fukao, Toshiyuki
A20单倍体不足（HA20）引起类似白塞氏病的炎性疾病；已经报道了许多病例，包括一些合并自身免疫性疾病的病例。本研究旨在通过多色流式细胞术分析淋巴细胞亚群，阐明HA20患者的免疫表型。先前在全国调查中诊断的HA20患者通过其细胞亚群进行比较。共分析调节性T细胞（Tregs）、双阴性T细胞（DNTs）、滤泡性辅助性T细胞（TFHs）等27项指标，并与0-1岁、2-6岁、7-19岁和>= 20岁4个年龄组的参考值进行比较。在所有年龄段，HA20患者的Tregs与年龄匹配的对照者有串联增加的趋势。此外，>= 20岁的患者与对照组相比，DNTs增加，而年轻患者的TFHs显著增加。在HA20患者中，DNTs和TFHs的增加可能促进自身免疫性疾病的发展。
Haploinsufficiency of A20 (HA20) causes inflammatory disease resembling Behcet's disease; many cases have been reported, including some that are complicated with autoimmune diseases. This study aims to clarify the immunophenotype of patients with HA20 by analyzing lymphocyte subsets using multicolor flow cytometry. The patients with HA20 previously diagnosed in a nationwide survey were compared by their cell subpopulations. In total, 27 parameters including regulatory T cells (Tregs), double-negative T cells (DNTs), and follicular helper T cells (TFHs) were analyzed and compared with the reference values in four age groups: 0-1, 2-6, 7-19, and >= 20 years. The Tregs of patients with HA20 tended to increase in tandem with age-matched controls at all ages. In addition, patients >= 20 years had increased DNTs compared with controls, whereas TFHs significantly increased in younger patients. In HA20 patients, the increase in DNTs and TFHs may contribute to the development of autoimmune diseases.