免疫缺陷病(Immunodeficiency Discase)是一组由于免疫活性细胞在发生、分化或代谢等方面存在异常而引起的一种或多种免疫功能不全或紊乱的综合征。其发病率与小儿时期之白血病相当。 临床上以反复感染为主征,但也易伴有自身稳定和免疫监督功能的异常,因而常伴发自身免疫性疾病,过敏性疾病及恶性肿瘤。为了加强对免疫缺陷病的认识,现将我院76年4月至83年4月经病理证实的原发性 Immunodeficiency Discase is a group of one or more immunocompromised or disorganized syndromes caused by aberrant occurrence, differentiation or metabolism of immunocompetent cells. Its incidence is comparable to childhood leukemia. Clinically, the main symptoms of recurrent infections, but also easily accompanied by its own stability and immune surveillance abnormalities, which often accompanied by autoimmune diseases, allergic diseases and malignant tumors. In order to strengthen the understanding of immunodeficiency disease, now our hospital from April 76 to April 83 pathologically confirmed by the primary