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免疫缺陷病是指由于先天发育不全(原发性)或后天受到损害(继发性),导致人体的一个或几个免疫机制障碍而发生的疾病。这类病人的临床表现主要是:(1)对感染具有高度的易感性;(2)容易发生自身免疫性疾病;(3)癌症的发病率较正常人群增高。其中尤以连绵不断的感染为免疫缺陷病的特征性表现。近年来,随着对免疫缺陷病认识的不断提高,在诊治方面也有了卓有成效的进展。人们发现即使是原发性免疫缺陷病也并不象以往认
Immunodeficiency disease is a disease that occurs as a result of one or several disturbances in the immune system due to congenital hypoplasia (primary) or acquired (secondary). The clinical manifestations of such patients are mainly: (1) a high degree of susceptibility to infection; (2) prone to autoimmune diseases; (3) the incidence of cancer is higher than the normal population. Among them, the continuous infection is the characteristic manifestation of immunodeficiency disease. In recent years, with the continuous improvement of understanding of immunodeficiency disease, there have been fruitful progress in diagnosis and treatment. It was found that even the primary immunodeficiency disease is not recognized as before