免疫缺陷病是指因免疫活性细胞和免疫活性分子 (包括细胞因子 ,细胞膜表面标记和粘附分子 )发生缺陷引起的免疫反应缺如或降低 ,导致机体抗感染免疫功能低下的一组临床综合征。因遗传因素如染色体异常或基因突变所致的免疫缺陷病称为原发性免疫缺陷病 (ｐｒｉ ｍａｒｙｉｍｍｕ Immunodeficiency disease refers to a group of clinical syndromes caused by lack of or decrease of immune response due to defects in immunocompetent cells and immunologically active molecules (including cytokines, cell membrane surface markers and adhesion molecules), which leads to the body’s anti-infective immune dysfunction . Immunodeficiency disease due to genetic factors such as chromosomal abnormalities or genetic mutations is called primary immunodeficiency disease (pri maryimmu