Silvestris N, Silvestris F, Russo S, Dammacco F
Dipartimento di Scienze Biomediche e Oncologia Umana, Università, Bari.
Recenti Prog Med. 1996 Dec;87(12):616-22.
Common variable immunodeficiency (CVI) is a heterogeneous syndrome characterized by defective antibody formation, resulting in abnormally low serum immunoglobulin levels. Clinical presentation usually includes recurrent infections of the respiratory tract, mostly induced by capsular bacteria. Patients are also highly prone to Giardia lamblia infections and related gastrointestinal disorders, as well as to a variety of autoimmune diseases which appear in approximately 20% of them. In addition, CVI can be frequently associated with a non-Hodgkin lymphoma or gastric carcinoma. In spite of its relatively frequent occurrence, the pathogenesis of CVI still remains poorly defined. In this review the authors describe clinical features, immunological abnormalities and replacement treatment with intravenous immunoglobulins of this antibody deficiency syndrome.
普通可变免疫缺陷（CVI）是一种异质性综合征，其特征为抗体形成缺陷，导致血清免疫球蛋白水平异常降低。临床表现通常包括呼吸道反复感染，主要由荚膜细菌引起。患者还极易感染蓝氏贾第鞭毛虫及相关胃肠道疾病，以及约20%的患者会出现的各种自身免疫性疾病。此外，CVI常与非霍奇金淋巴瘤或胃癌相关。尽管其发病率相对较高，但CVI的发病机制仍不清楚。在这篇综述中，作者描述了这种抗体缺陷综合征的临床特征、免疫异常以及静脉注射免疫球蛋白替代治疗。
