Arduini Serena, Dunne Jean, Conlon Niall, Feighery Conleth, Doherty Derek G
Discipline of Immunology, School of Medicine, Trinity College Dublin, Dublin, Ireland.
Department of Immunology, St. James's Hospital, Dublin, Ireland.
Clin Immunol. 2017 Mar;176:23-30. doi: 10.1016/j.clim.2016.12.002. Epub 2016 Dec 21.
Common variable immunodeficiency (CVID) is a primary immunoglobulin deficiency characterized by recurrent infections and complications, including autoimmunity, enteropathy, polyclonal lymphocytic infiltration or lymphoid malignancy. Innate T cells can support B cell maturation and antibody production. We investigated the numbers, phenotypes and functions of circulating B cell, γδ T cell, invariant natural killer T (iNKT) cell and mucosal-associated invariant T (MAIT) cell subsets in 23 CVID patients and 27 healthy controls. Switched-memory B cells and plasmablasts were depleted in CVID patients (p<0.0001). γδ T cells were found at normal numbers, but iNKT and MAIT cells were depleted (p<0.0001 and p<0.002). MAIT cells were especially low in patients with complicated CVID (p<0.05). MAIT cells from patients appeared more activated and more frequently produced interleukin-17A, interleukin-22 and tumor necrosis factor-α than MAIT cells from healthy subjects in vitro. Thus, MAIT cell depletion and activation may contribute to immunodeficiency and complications associated with CVID.
常见变异型免疫缺陷（CVID）是一种原发性免疫球蛋白缺乏症，其特征为反复感染及并发症，包括自身免疫、肠病、多克隆淋巴细胞浸润或淋巴恶性肿瘤。固有T细胞可支持B细胞成熟及抗体产生。我们调查了23例CVID患者及27例健康对照者循环B细胞、γδ T细胞、不变自然杀伤T（iNKT）细胞和黏膜相关不变T（MAIT）细胞亚群的数量、表型及功能。CVID患者的转换记忆B细胞和浆母细胞减少（p<0.0001）。γδ T细胞数量正常，但iNKT细胞和MAIT细胞减少（p<0.0001和p<0.002）。复杂型CVID患者的MAIT细胞尤其少（p<0.05）。与健康受试者的MAIT细胞相比，患者的MAIT细胞在体外似乎更易激活，且更频繁地产生白细胞介素-17A、白细胞介素-22和肿瘤坏死因子-α。因此，MAIT细胞减少和激活可能导致与CVID相关的免疫缺陷及并发症。
