Salzer Ulrich, Grimbacher Bodo
Department of Clinical Immunology and Rheumatology, Medical Center, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany.
Semin Immunol. 2006 Dec;18(6):337-46. doi: 10.1016/j.smim.2006.07.004. Epub 2006 Oct 4.
Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. CVID is characterized by the sequelae of an antibody deficiency syndrome: an impaired terminal B cell differentiation results in hypogammaglobulinemia and susceptibility to recurrent infections by encapsulated bacteria. The clinical course of CVID is complicated by a plethora of systemic immunopathology, including autoimmunity, lymphoproliferation, malignancy and sarcoid-like granulomas. Phenotypic and functional studies in CVID patients revealed multiple abnormalities within the innate and adaptive immune system. The recent description of monogenic defects in ICOS, TACI and CD19 focussed our interest to an impaired T cell-B cell collaboration within the germinal center and intrinsic B cell defects as possible explanations for the etiology of CVID.
普通变异型免疫缺陷（CVID）是成人中最常见的有症状的原发性免疫缺陷病。CVID的特征是抗体缺陷综合征的后遗症：终末B细胞分化受损导致低丙种球蛋白血症，并易受包膜细菌反复感染。CVID的临床病程因多种全身性免疫病理学情况而复杂化，包括自身免疫、淋巴细胞增殖、恶性肿瘤和结节病样肉芽肿。对CVID患者的表型和功能研究揭示了先天性和适应性免疫系统内的多种异常。最近对ICOS、TACI和CD19单基因缺陷的描述使我们将兴趣集中在生发中心内T细胞与B细胞协作受损以及B细胞内在缺陷上，将其作为CVID病因的可能解释。
