选择性IgA缺乏症(Selective IgAdeficiency)是指体液IgA含量极低,而其它免疫球蛋白含量正常或升高的一种体液免疫缺陷病。本症首由West等于1962年报告,是最多见的一种原发性体液免疫缺陷病。在欧美,约500～700正常人群中有一例。过去认为原发性兔疫缺陷病是少见病,随着免疫学的发展,这种观点正在改变。 IgA的分布及产生 IgA约占血清免疫球蛋白总量15％(5～25％),成人血清平均浓度为250毫克/分升。 IgA有单体、双体、三体三种结构形式,它们在体内的分布不同。血清 Selective IgAdeficiency is a humoral immune deficiency disorder with normal or elevated IgA levels in other body fluids. The first symptom of West equal to the 1962 report, is the most common of a primary humoral immune deficiency disease. In Europe and the United States, there are about 500 to 700 normal people. In the past, primary idiopathic STDs were considered as infrequent diseases. With the development of immunology, this view is changing. IgA distribution and IgA production accounts for about 15% (5-25%) of the total serum immunoglobulin, the average serum concentration of 250 mg / dL. IgA has monomer, dyad, trisomy three kinds of structural forms, their distribution in the body is different. serum