普通变异型免疫缺陷病(Common va-riable immunodeficiency,CVID)是一组比较笼统的疾病范畴,根据WHO专家会议的推荐,将凡不能列入已确定的原发性免疫缺陷病(PID)的其它类型均归于本病。“普通”是指本病为常见的PID类型,具有较高的发病率。“变异”的含义很广,包括发病年龄的不定,病因发病机理的多样以及临床表现的易变。由于缺乏相应的动物模型,因而在较长的时间内对本病的本质一直认识不清。晚近,由于对淋巴细胞亚群研究的不断深入,尤其是应用单克隆技术和淋巴细胞酶生化代谢研究的开展,对本病的认识才日趋完善,现认为CVID的发病除了与原发性B细胞缺陷有关外,还与T细胞调控网络的异常密切有关,并且分出了以B细胞缺陷为主和以T细胞缺陷为主的两类基本的CVID表 Common va-rible immunodeficiency (CVID) is a relatively general category of disease. According to the recommendations of the WHO expert meeting, all patients who can not be included in the identified primary immunodeficiency disease (PID) Type are attributed to the disease. “Normal” refers to the disease as a common PID type, with a high incidence. “Variation” has a wide range of meaning, including variable age of onset, diverse pathogenesis and variable clinical manifestations. Due to the lack of corresponding animal models, the nature of the disease has not been fully understood over a long period of time. Recently, due to the continuous research on lymphocyte subsets, especially the application of monoclonal technology and lymphocyte enzymatic biochemical metabolism research, understanding of the disease is only getting better, it is thought that the incidence of CVID except with primary B cells Defects, but also with the T cell regulatory network is closely related to the abnormal and divided into two types of basic BID defects and T cell defects based CVID table