Immunophenotyping of A20 haploinsufficiency by multicolor flow cytometry.
Adolescent
Adult
Autoimmune Diseases
/ immunology
Behcet Syndrome
/ immunology
Child
Child, Preschool
Female
Flow Cytometry
/ methods
Haploinsufficiency
/ immunology
Humans
Immunophenotyping
/ methods
Infant
Male
Phenotype
T-Lymphocytes, Helper-Inducer
/ immunology
T-Lymphocytes, Regulatory
/ immunology
Young Adult
Autoimmunity
Double-negative T cell
Follicular helper T cell
Haploinsufficiency of A20
Regulatory T cell
TNFAIP3
Journal
Clinical immunology (Orlando, Fla.)
ISSN: 1521-7035
Titre abrégé: Clin Immunol
Pays: United States
ID NLM: 100883537
Informations de publication
Date de publication:
07 2020
07 2020
Historique:
received:
27
12
2019
revised:
27
03
2020
accepted:
22
04
2020
pubmed:
27
4
2020
medline:
2
2
2021
entrez:
27
4
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32335289
pii: S1521-6616(19)30712-0
doi: 10.1016/j.clim.2020.108441
pii:
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
108441Informations de copyright
Copyright © 2020 Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of Competing Interest The authors declare no conflicts of interest.