Abstract
BACKGROUND: Common variable immunodeficiency (CVID) is characterized by low immunoglobulin G and IgA/IgM, decreased switched memory B cells, impaired response to vaccine, and an increased susceptibility to infections and autoimmunity. T(FH) cells play an important role in germinal center reaction where it supports isotype switching, somatic hypermutation, generation of memory B cells, and differentiation of B cells to plasma cells. The objective was to study the distribution of three subsets of T(FH) cells and their relationship with autoimmune diseases associated with CVID. METHODS: T(FH) cells have been divided into T(FH) 1 (interleukin 21 [IL-21] and interferon γ), T(FH) 2 (IL-21 and IL-4), and T(FH) 17 (IL-21 and IL-17) cells. Mononuclear cells from 25 patients with CVID and age and gender-matched controls were stained with various monoclonal antibodies (anti-CD4 APC, anti-CXCR5 FITC, anti-CCR6 PerCP, and anti-CXCR3 PE) and isotype controls and analyzed for T(FH) 1 (CD4(+) CXCR5(+) CXCR3(+) CCR6(-) ), T(FH) 2 (CD4(+) CXCR5(+) CXCR3(-) CCR6(-) ), and T(FH) 17 (CD4(+) CXCR5(+) CXCR3(-) CCR6(+) ) cells by multicolor flow cytometry. Twenty thousand cells were acquired and analyzed by FlowJo software. Statistical analysis of comparison of patients and healthy controls was performed by paired t test using PRISM 7 software. RESULTS: T(FH) 2 and T(FH) 17 cells subpopulations of T(FH) cells were significantly decreased (P < .003 and P < .006, respectively) in CVID as compared with controls. No significant difference was observed in any of T(FH) cell subpopulations between CVID with and those without autoimmunity group. CONCLUSION: Alterations in T(FH) cell subpopulation may play a role in defects in B cell compartment in CVID.