选择性IgA缺乏症是一种最常见的原发性免疫缺陷病。自1962年West等首次报道以来,病例报道逐渐增多。国内外学者对其流行病学、诊断、发病机理、临床及免疫学表现、相关疾病、治疗及预后等进行了探讨。由于本症具有多因异质性,在判断时常遇到一些困难,现就以下几方面进行讨论。 Selective IgA deficiency is one of the most common primary immunodeficiency disorders. Since the first report by West et al. In 1962, case reports have been gradually increasing. Domestic and foreign scholars on its epidemiology, diagnosis, pathogenesis, clinical and immunological findings, related diseases, treatment and prognosis were discussed. Because of this disease has many causes of heterogeneity, often encountered some difficulties in the judgment, the following aspects are discussed.