Etzioni Amos
Meyer Children's Hospital, Haifa and the B. Rappaport Faculty of Medicine, Technion, Haifa, 36019, Israel.
Autoimmun Rev. 2003 Oct;2(6):364-9. doi: 10.1016/s1568-9972(03)00052-1.
Immunodeficiency and autoimmune phenomena may occur concomitantly in the same individual. Many immune deficiency syndromes, mainly humoral defects, are associated with autoimmune disorders. Hematological manifestations, such as thrombocytopenia and hemolytic anemia, are the most common presentation, but many other autoimmune mediated conditions have also been described. Persistent antigen stimulation, due to an inherently defective immune system ability to eradicate pathogenesis is the primary cause leading to autoimmunity in patients with primary immunodeficiency states. Other factors leading to the increase incidence of autoimmune manifestion will be discussed in the present review. Treatment with intravenous gammagluobuilin may ameliorate the autoimmune disorder and bone marrow transplantation can cure both conditions.
免疫缺陷和自身免疫现象可能在同一个体中同时出现。许多免疫缺陷综合征，主要是体液缺陷，与自身免疫性疾病相关。血液学表现，如血小板减少和溶血性贫血，是最常见的表现，但也有许多其他自身免疫介导的病症被描述。由于免疫系统根除病原体的能力固有缺陷导致的持续抗原刺激是导致原发性免疫缺陷状态患者自身免疫的主要原因。导致自身免疫表现发生率增加的其他因素将在本综述中讨论。静脉注射免疫球蛋白治疗可能改善自身免疫性疾病，骨髓移植可以治愈这两种病症。
