Common variable immunodeficiency:report of 12 cases and review of literature
- VernacularTitle:普通变异型免疫缺陷病12例临床分析并文献复习
- Author:
Wei Lü
;
Zhengyin LIU
;
Taisheng LI
- Publication Type:Journal Article
- Keywords:
Immunoglobulins;
Common variable immunodeficieney
- From:
Chinese Journal of Internal Medicine
2008;47(5):378-381
- CountryChina
- Language:Chinese
-
Abstract:
Objective 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.
