PDF(Pubmed)
Abstract：
OBJECTIVE: Common Variable Immunodeficiency (CVID) is characterized by hypogammaglobulinemia and failure of specific antibody production due to B-cell defects. However, studies have documented various T-cell abnormalities, potentially linked to viral complications. The frequency of Cytomegalovirus (CMV) replication in CVID cohorts is poorly studied. To address this gap in knowledge, we set up an observational study with the objectives of identifying CVID patients with active viraemia (CMV, Epstein-Barr virus (EBV)), evaluating potential correlations with immunophenotypic characteristics, clinical outcome, and the dynamic progression of clinical phenotypes over time.
METHODS: 31 CVID patients were retrospectively analysed according to viraemia, clinical and immunologic characteristics. 21 patients with non CVID humoral immunodeficiency were also evaluated as control.
RESULTS: Active viral replication of CMV and/or EBV was observed in 25% of all patients. CMV replication was detected only in CVID patients (16%). CVID patients with active viral replication showed reduced HLA-DR+ NK counts when compared with CMV-DNA negative CVID patients. Viraemic patients had lower counts of LIN-DNAMbright and LIN-CD16+ inflammatory lymphoid precursors which correlated with NK-cell subsets. Analysis of the dynamic progression of CVID clinical phenotypes over time, showed that the initial infectious phenotype progressed to complicated phenotypes with time. All CMV viraemic patients had complicated disease.
CONCLUSIONS: Taken together, an impaired production of inflammatory precursors and NK activation is present in CVID patients with active viraemia. Since \"Complicated\" CVID occurs as a function of disease duration, there is need for an accurate evaluation of this aspect to improve classification and clinical management of CVID patients.
METHODS: 31 CVID patients were retrospectively analysed according to viraemia, clinical and immunologic characteristics. 21 patients with non CVID humoral immunodeficiency were also evaluated as control.
RESULTS: Active viral replication of CMV and/or EBV was observed in 25% of all patients. CMV replication was detected only in CVID patients (16%). CVID patients with active viral replication showed reduced HLA-DR+ NK counts when compared with CMV-DNA negative CVID patients. Viraemic patients had lower counts of LIN-DNAMbright and LIN-CD16+ inflammatory lymphoid precursors which correlated with NK-cell subsets. Analysis of the dynamic progression of CVID clinical phenotypes over time, showed that the initial infectious phenotype progressed to complicated phenotypes with time. All CMV viraemic patients had complicated disease.
CONCLUSIONS: Taken together, an impaired production of inflammatory precursors and NK activation is present in CVID patients with active viraemia. Since \"Complicated\" CVID occurs as a function of disease duration, there is need for an accurate evaluation of this aspect to improve classification and clinical management of CVID patients.
摘要：
目的：普通可变免疫缺陷（CVID）的特征是低丙种球蛋白血症和B细胞缺陷导致的特异性抗体产生失败。然而,研究记录了各种T细胞异常，可能与病毒并发症有关。CVID队列中巨细胞病毒（CMV）复制的频率研究甚少。为了解决这个知识差距，我们建立了一项观察性研究，目的是识别患有活动性病毒血症的CVID患者（CMV，爱泼斯坦-巴尔病毒（EBV），评估与免疫表型特征的潜在相关性，临床结果，以及临床表型随时间的动态进展。
方法：31例CVID患者根据病毒血症进行回顾性分析，临床和免疫学特征。还评估了21例非CVID体液免疫缺陷患者作为对照。
结果：在所有患者中25％观察到CMV和/或EBV的活跃病毒复制。CMV复制仅在CVID患者中检测到(16%)。与CMV-DNA阴性CVID患者相比，病毒复制活跃的CVID患者显示HLA-DRNK计数降低。病毒血症患者的LIN-DNAMbright和LIN-CD16炎性淋巴前体计数较低，与NK细胞亚群相关。分析CVID临床表型随时间的动态进展，表明初始感染表型随时间进展为复杂表型。所有CMV病毒血症患者均患有复杂疾病。
结论：综合来看，在患有活动性病毒血症的CVID患者中存在炎性前体的产生和NK激活受损。由于“复杂”CVID作为疾病持续时间的函数而发生，需要对这方面进行准确评估,以改善CVID患者的分类和临床管理.
方法：31例CVID患者根据病毒血症进行回顾性分析，临床和免疫学特征。还评估了21例非CVID体液免疫缺陷患者作为对照。
结果：在所有患者中25％观察到CMV和/或EBV的活跃病毒复制。CMV复制仅在CVID患者中检测到(16%)。与CMV-DNA阴性CVID患者相比，病毒复制活跃的CVID患者显示HLA-DRNK计数降低。病毒血症患者的LIN-DNAMbright和LIN-CD16炎性淋巴前体计数较低，与NK细胞亚群相关。分析CVID临床表型随时间的动态进展，表明初始感染表型随时间进展为复杂表型。所有CMV病毒血症患者均患有复杂疾病。
结论：综合来看，在患有活动性病毒血症的CVID患者中存在炎性前体的产生和NK激活受损。由于“复杂”CVID作为疾病持续时间的函数而发生，需要对这方面进行准确评估,以改善CVID患者的分类和临床管理.
