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巨细胞病毒病在普通变异性免疫缺陷患者中的表现:三例病例报告。

Cytomegalovirus disease in patients with common variable immunodeficiency: three case reports.

机构信息

Institute of Clinical Immunology and Allergy, University Hospital Hradec Kralove, Faculty of Medicine, Charles University in Prague, Hradec Kralove, Czech Republic.

出版信息

Int Arch Allergy Immunol. 2014;163(1):69-74. doi: 10.1159/000355957. Epub 2013 Nov 16.

DOI:10.1159/000355957
PMID:24247002
Abstract

Common variable immunodeficiency (CVID) is the most frequent clinically relevant primary immunodeficiency and shows enormous heterogeneity in clinical presentation. Despite clinical immunodeficiency, opportunistic infections are not a typical manifestation of CVID. A retrospective study of 32 patients followed up for 335 patient-years was performed to determine the frequency of cytomegalovirus (CMV) disease. Symptomatic CMV infection was documented in 3 CVID patients. Patients No. 1 and 2 suffered from CMV pneumonia, with complications due to atypical mycobacteriosis in patient No. 1. Patient No. 3 suffered from CMV enteritis. A history of cancer and chronic hepatitis C infection (patient No. 1), immunosuppressive therapy for interstitial lung disease (patient No. 2) and serious enteropathy complicated with malnutrition (patient No. 3) may have contributed to the complications despite only mild abnormalities in T-cell subpopulations. The direct detection of CMV in bronchoalveolar lavage, stool or tissue samples was the most beneficial diagnostic laboratory method, whereas the detection of CMV DNA in blood did not produce positive results. Adequate treatment of CMV disease led to significant clinical improvement in all 3 patients. The frequency of CMV disease appears to be higher than previously described. In our experience, the probability of opportunistic infections in CVID patients increases with secondary comorbidities and their management.

摘要

普通变异性免疫缺陷症(CVID)是最常见的临床相关原发性免疫缺陷症,其临床表现存在巨大的异质性。尽管存在临床免疫缺陷,但机会性感染并非 CVID 的典型表现。对 32 例患者进行了 335 患者年的回顾性研究,以确定巨细胞病毒(CMV)疾病的频率。3 例 CVID 患者确诊有症状性 CMV 感染。患者 1 和 2 患有 CMV 肺炎,患者 1 因非典型分枝杆菌病而出现并发症。患者 3 患有 CMV 肠炎。尽管 T 细胞亚群仅有轻度异常,但癌症和慢性丙型肝炎感染史(患者 1)、间质性肺病的免疫抑制治疗(患者 2)以及严重的伴有营养不良的肠炎(患者 3)可能导致了并发症。直接检测支气管肺泡灌洗液、粪便或组织样本中的 CMV 是最有益的诊断实验室方法,而血液中 CMV DNA 的检测并未产生阳性结果。对 CMV 疾病的充分治疗使所有 3 例患者的临床状况都得到了显著改善。CMV 疾病的频率似乎高于之前的描述。根据我们的经验,继发合并症及其管理会增加 CVID 患者发生机会性感染的概率。

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