Clinical immunology (Orlando, Fla.) · 2020
Immunophenotyping of A20 haploinsufficiency by multicolor flow cytometry.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Kadowaki Tomonori, Ohnishi Hidenori, Kawamoto Norio, Kadowaki Saori, Hori Tomohiro, Nishimura Kenichi, Kobayashi Chie, Shigemura Tomonari, Ogata Shohei, Inoue Yuzaburo, Hiejima Eitaro, Izawa Kazushi, Matsubayashi Tadashi, Matsumoto Kazuaki, Imai Kohsuke, Nishikomori Ryuta, Ito Shuichi, Kanegane Hirokazu, Fukao Toshiyuki
Аннотация
Haploinsufficiency of A20 (HA20) causes inflammatory disease resembling Behçet'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.