Common variable immunodeficiency (CVID) is characterized by low levels of circulating immunoglobulins and defects in B cell maturation leading to an increased susceptibility to infections. Some patients develop complications such as autoimmune diseases, enteropathy, and lymphoproliferation, resulting in higher morbidity and mortality. Follicular helper T (Tfh) cells are specialized in helping B cell differentiation into Ig-producing cells. Three subsets have been described, namely non B-cell helper Tfh1 and the two B-helper cell subsets Tfh2 and Tfh17. We determined that circulating Tfh cells were elevated in CVID patients and skewed toward a Tfh1 profile. Interestingly, elevated levels of Tfh1 cells were significant only in patients harboring non-infectious complications regardless of the type of complication and inversely correlated with switched memory B cells. Moreover, CXCR3⁺ cells are increased in splenic CVID germinal centers. Our observations suggest that the altered balance in Tfh subsets in CVID is linked to a more severe disease.

常见的可变免疫缺陷症（CVID）的特征是循环免疫球蛋白水平低和B细胞成熟缺陷，导致对感染的敏感性增加。一些患者出现并发症，例如自身免疫性疾病，肠病和淋巴增生，导致更高的发病率和死亡率。滤泡辅助性T（T fh）细胞专门用于帮助B细胞分化为产生Ig的细胞。已经描述了三个子集，即非B细胞辅助细胞T fh 1和两个B帮助细胞子集T fh 2和T fh17。我们确定CVID患者的循环T fh细胞升高并且偏向T fh1个个人资料。有趣的是，无论并发症的类型如何，T fh 1细胞的升高水平仅在具有非感染性并发症的患者中才有意义，并且与开关记忆B细胞呈负相关。此外，CXCR3 ⁺细胞在脾CVID生发中心增加。我们的观察结果表明，CVID中T fh亚群平衡的改变与更严重的疾病有关。