普通变异型免疫球蛋白缺乏症5例并文献复习

目的旨在提高对临床表现各异的普通变异型免疫球蛋白缺乏症(common variable im-munodeficiency,CVID)的认识,以减少临床漏诊和误诊率。方法回顾我院5例CVID患者的诊治经过,并对该病的发病机制、免疫学改变和临床诊治等研究进展进行文献复习。结果5例患者分别以伴或不伴有发热、消化道、呼吸道症状就诊,最终检测免疫球蛋白明显降低而确诊,予丙种球蛋白及相应抗感染治疗后好转出院。结论CVID为原发性免疫缺陷病的一种,成年人发病多见,临床以反复感染为主要特征,尤以消化系统、呼吸系统为突出表现,可伴有自身免疫病和恶性肿瘤,血清免疫球蛋白明显减低有助于确诊。治疗上采取丙种球蛋白替代治疗。

A report of 5 cases of common variable immunodeficiency with related literature review

Objective To increase the understanding for common variable immunodeficiency disease (CVID) with different clinical manifestations in order to decrease the clinical missed diagnosis and misdiagnosis rate.Methods The diagnostic and therapeutic courses of 5 cases of CVID in our hospital were retrospectively analyzed,and the related literatures about pathogenesis, clinical manifestations, diagnosis and treatment of CVID were reviewed.Results Five patients came to the hospital for consultation with the chief complains of digestive or respiratory symptoms, with or without fever.CVID was diagnosed when hypo-immunoglobulin were found after a series of examinations.The patients were treated by gamma globulin and anti-infection drugs,and the patients recovered and were discharged from hospital.Conclusions CVID is a heterogeneous immunodeficiency syndrome characterized by hypo-immunoglobulinemia, recurrent bacterial infection in respiratory and/or digestive system,accompanying with autoimmune diseases or malignant tumor. Hypo-immunoglobulin is the main evidence of diagnosis. Introvenously administered gamma globulin is an effective therapy.