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成人原发性免疫缺陷:常见可变免疫缺陷——临床表现、免疫和遗传缺陷、治疗

[Primary immunodeficiency in adults: common variable immunodeficiency--clinical manifestations, immunological and genetic defects, treatment].

出版信息

Ter Arkh. 2011;83(10):49-54.

Abstract

The most prevalent form of primary immunodeficiency with a total defect of antibody production in adults is common variable immunodeficiency (CVID). Compared to other forms of primary immunodeficiency, CVID is characterized by later onset of clinical manifestations represented by infectious, autoimmune and malignant diseases. To avoid development of complications and patient incapacitation, it is necessary to make an early diagnosis and initiate regular replacement therapy with intravenous immunoglobulins.

摘要

成人中抗体产生完全缺陷的最常见原发性免疫缺陷形式是普通可变免疫缺陷(CVID)。与其他形式的原发性免疫缺陷相比,CVID的特点是临床表现出现较晚,以感染性、自身免疫性和恶性疾病为代表。为避免并发症的发生和患者失能,有必要尽早诊断并开始定期静脉注射免疫球蛋白替代治疗。

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