近些年来,由于诊断水平的提高,原发性免疫缺陷病的发现与报告日益增多。重度联合免疫缺陷病(简称联合缺陷)作为原发性免疫缺陷之一种,是同时有细胞免疫缺陷和体液免疫映陷的先天性遗传性疾病。患儿出生后几个月内即发病,进行性发展,常在较短时间死亡。到1973年,联合缺陷已报道200余例。本教研组最近两年内尸检本病3例。鉴于国内对本病报道尚少,故予以报道,并复 In recent years, due to the increased diagnostic level, the discovery and report of the primary immunodeficiency disease is increasing day by day. Severe combined immunodeficiency disease (referred to as the combined defect) as a primary immune deficiency, is a congenital hereditary diseases that both cellular immunodeficiency and humoral immunity map. Children born within a few months after onset, progressive development, often died in a relatively short period of time. By 1973, more than 200 joint defects were reported. The teaching and research group autopsy in the last two years the disease in 3 cases. In view of the domestic reports of the disease is still small, it is reported, and complex