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由先天性遗传所致的免疫缺陷在临床上表现为多种疾病。主要有细胞免疫缺陷和体液免疫缺陷两大类。而细胞介导的免疫缺陷与抗体介导的免疫缺陷临床表现很不相同。前者为患儿对机会感染有明显的易感性及恶性肿瘤发生率增高,后者则表现为反复化脓性感染。本文将介绍原发性细胞免疫缺陷在临床上所表现的主要几种疾病。重型联合免疫缺陷瘸(SCID) Sevare combined immunodeficiency(SCID)是细胞介导的免疫缺陷病中最常见的一种形式,包括T、B两类细胞缺乏为特征的一组综合征。因缺乏干细胞,阻碍T细胞成熟或者细胞膜发育异常而引起T、B淋
Immune deficiencies caused by congenital hereditary clinical manifestations of a variety of diseases. There are two major categories of cellular immunodeficiency and humoral immune deficiency. However, cell-mediated immunodeficiency and antibody-mediated immune deficiency clinical manifestations are very different. The former for children with opportunistic infections have obvious susceptibility and the incidence of malignant tumors increased, the latter showed repeated purulent infection. This article will introduce the primary cellular immune deficiency in the clinical manifestations of several major diseases. Severe Combined Immunodeficiency (SCID) Sevare combined immunodeficiency (SCID) is the most common form of cell-mediated immune deficiency disease and includes a group of syndromes characterized by a deficiency of T and B cells. Due to lack of stem cells, hinder the maturation of T cells or abnormal cell membrane caused by T, B lymph