Common variable immunodeficiency: Clinical and immunological features of 248 patients
Common variable immunodeficiency: Clinical and immunological features of 248 patients
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DOI：
10.1006/clim.1999.4725
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发表时间：
1999-07-01
影响因子：
8.6
通讯作者：
Bodian, C
中科院分区：
文献类型：
--
作者：
Cunningham-Rundles, C;Bodian, C
常见变异型免疫缺陷（CVI）是一种原发性免疫缺陷病，其特征是血清免疫球蛋白降低和异质性临床特征。在这些研究中，我们描述了248例连续转诊的年龄范围为3-79岁的CVI患者的临床和免疫学状态，这些患者已随访1-25年。症状发作时的中位年龄男性为23岁，女性为28岁;诊断为CVI的平均年龄男性为29岁，女性为33岁。40%的患者有受损的T细胞增殖到一个或多个有丝分裂原;淋巴细胞转化为有丝分裂原是直接相关的血清IgG水平。所有年龄段的女性血清IgM水平均高于男性。诊断为CVI后20年的生存率男性为64%，女性为67%，而男性和女性的预期人群生存率分别为92%和94%。在此期间，与死亡率相关的参数是血清IgG水平较低，T细胞对植物血凝素的反应较差，特别是外周B细胞百分比较低（P < 0.006）。(C)北京：科学出版社.
Common variable immunodeficiency (CVI) is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. In these studies we describe the clinical and immunological status of 248 consecutively referred CVI patients of age range 3-79 years who have been followed for a period of 1-25 years. The median age at the time of onset of symptoms was 23 years for males and 28 years for females; the mean age at which the diagnosis of CVI was made was 29 years for males and 33 years for females. Forty percent of patients had impaired T cell proliferation to one or more mitogens; lymphocyte transformation to mitogens was directly related to the level of the serum IgG. Females at all ages had higher levels of serum IgM than males. Survival 20 years after diagnosis of CVI was 64% for males and 67% for females, compared to the expected 92% population survival for males and 94% for females. Parameters associated with mortality in this period were lower levels of serum IgG, poorer T cell responses to phytohemagglutinin, and, particularly, a lower percentage of peripheral B cells (P < 0.006). (C) 1999 Academic Press.