摘要目的 探讨普通变异型免疫缺陷病(comnlon variable immunodeficiency,CVID)的临床特点,提高临床医师对该病的认识.方法 回顾性分析北京协和医院1990年1月-2007年3月确诊为CVID的12例患者临床资料、实验室检查、治疗和预后.结果 12例患者男女比例为2:1,发病年龄为(26±9)岁,发病到确诊时间平均18个月.主要以反复发热(67.0%)、反复咳嗽、咳痰(58.3%)、腹泻(41.6%)为临床表现,实验窜改变有贫血(58.3%)和白细胞减少(58.3%).全部病例依靠检测到免疫球蛋白降低(IgG、IgM、IgA)确诊.9例病人的淋巴细胞检测显示,B细胞和CD4+ T 细胞均低下,其中7例病人CD4/CD8比例倒置.有10例病人接受丙种球蛋白治疗,临床症状均好转.结论 CVID为原发性免疫缺陷病的一种,成年发病多见,临床以消化系统、呼吸系统的反复感染为突出表现,可伴有自身免疫病和肿瘤.确诊依靠检测血清免疫球蛋白.治疗上采取丙种球蛋白替代治疗,大多预后较好.

abstractsObjective To study the clinical features of a heterogeneous immunodeficiency disease,common vailable immunodeficiency(CVID),and to enhance the understanding of it.Methods 12 cases of CVID treated in Peking Union Medical College Hospital from January 1990 to March 2007 were analyzed retrospectively,including the clinical characteristics,laboratory results,treatment and prognosis.Results Among the 12 patients,the ratio of male to female Was 2:1,the average onset age(26±9)years old and the median time from onset to diagnosis 18 months.The main symptoms were fever(with a percentage of 67.0%),recurrent Cough and expectoration(58.3%)and diarrhea(41.6%).Anemia and leukocytopenia were the common laboratory changes.All the cases were diagnosed due to the presence of hypo- immunoglobulinemia.The tests available for subtypes of lymphocytes in 9 patients showed that B cells and CD4+ T cells decreased obviously,with an inverse ratio of CD4/CD8,indicating T cell dysfunction.Clinical improvement was demonstrated after treatment with intravenously administered immunoglobulin(IVIG)in 10 cases.Conclusions CVID is a heterogeneous group of immunologic disorders of unknown etiology,characterized by impaired antibody responses and recurrent airway and/or gastrointestinal infection and accompanied with autoimmune diseases or cancer.Hypo-immunoglobulinemia is the main evidence of its diagnosis.IVIG as a replacement therapy is an effective way of management.