Introduction: One of the most frequent abnormalities of B cells in common variable immunodeficiency (CVID) is reduced number of class-switched memory B cells, suggesting an impaired germinal center response. Therefore, due to its pivotal role in regulating the development of humoral immunity, the objective of this study was to evaluate the role of circulating T follicular helper (cT_FH) and circulating T follicular regulatory (cT_FR) cells in the pathogenesis of CVID. Methods: cT_FH and cT_FR cells from CVID patients and healthy subjects were phenotypically characterized by flow cytometry. cT_FH and memory B cells from CVID patients and healthy subjects were isolated and cocultured. Results: Our results showed a reduced proportion of cT_FH17 cells in patients with CVID and an increased ratio of cT_FH/cT_FR cells in CVID patients with autoimmune diseases. Furthermore, the proportion of IL-21-producing cT_FH cells was directly related to the proportion of CD27⁺ IgD⁻ B cells. Interestingly, coculture assay showed that CVID-derived cT_FH cells are able to help memory B cells from healthy controls to produce immunoglobulins. Conclusions: The proportions of cT_FH17 and cT_FR cells are altered in CVID patients; however, the cT_FH function in assisting B cells to produce antibodies in vitro is preserved.
Int Arch Allergy Immunol

中文翻译：

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常见可变免疫缺陷性T滤泡细胞的表型和功能分析。

简介：在常见可变免疫缺陷症（CVID）中，B细胞最常见的异常现象之一是类别转换记忆B细胞的数量减少，这提示生发中心反应受损。因此，由于其在调节体液免疫发展中的关键作用，本研究的目的是评估循环T卵泡辅助细胞（cT _FH）和循环T卵泡调节细胞（cT _FR）在CVID发病机理中的作用。方法：利用流式细胞仪对CVID患者和健康受试者的cT _FH和cT _FR细胞进行表型鉴定。CT _FH分离并共培养来自CVID患者和健康受试者的记忆B细胞。结果：我们的结果显示，CVID患者的cT _FH 17细胞比例降低，而自身免疫性疾病的CVID患者的cT _FH / cT _FR细胞比例增加。此外，产生IL-21的cT _FH细胞的比例与CD27 ⁺ IgD ^- B细胞的比例直接相关。有趣的是，共培养分析表明，CVID衍生的cT _FH细胞能够帮助健康对照者记忆B细胞产生免疫球蛋白。结论： cT _FH 17和cT的比例CVID患者的_FR细胞发生改变；但是，保留了cT _FH协助B细胞在体外产生抗体的功能。
Int Arch过敏免疫