常见可变免疫缺陷
低丙种球蛋白血症
免疫学
免疫缺陷
原发性免疫缺陷
疾病
人口
免疫系统
医学
抗体
B细胞
发病机制
淋巴增殖性病變
生物
淋巴瘤
病理
环境卫生
作者
Corentin Le Saos-Patrinos,Séverine Loizon,Patrick Blanco,Jean‐François Viallard,Dorothée Duluc
标识
DOI:10.3389/fimmu.2020.00006
摘要
Common variable immunodeficiency is the most common clinical primary immunodeficiency in adults. Its hallmarks are hypogammaglobulinemia and compromised B-cell differentiation into memory or antibody-secreting cells leading to recurrent infections. This disease is heterogeneous, with some patients harboring multiple complications such as lymphoproliferative disorders, autoimmune manifestations, or granulomatous inflammation. The mechanisms leading to these complications remain elusive despite numerous associations found in the literature. For instance, although described as a B cell intrinsic disease, numerous abnormalities have been reported in other immune cell compartments. Here, we tuned our attention to follicular helper T cells, a CD4+ T cell population specialized in B cell help, considering the recent publications showing an involvement of these cells in CVID pathogenesis.
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