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Nijmegen breakage syndrome: Long-term monitoring of viral and immunological biomarkers in peripheral blood before development of malignancy.

机译:奈梅亨破坏综合症:在恶性肿瘤发生之前长期监测外周血中的病毒和免疫生物标记。

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摘要

Selected viruses and immune parameters were monitored in 57 patients with Nijmegen breakage syndrome as a proposed tool for early detection of changes preceding development of malignancy. The following parameters were analysed: (1) viral infections; (2) monoclonal proteins; and (3) B-cell and T-cell receptor gene rearrangements in peripheral blood lymphocytes. Viral infections were detected in 68.4% of patients with a predominance of EBV (63.2%), followed by HBV (19.2%) and HCV (8.8%). Monoclonal gammopathy detected in 38.6% of cases correlated with the presence of EBV DNA (p=0.002) and HCV RNA (p=0.04). Clonal Ig and/or TCR gene rearrangements occurred in 73.9% of patients. The presence of at least one of the studied parameters preceded the development of malignancy in 22 patients. Systematic PCR analysis for viral infections and Ig/TCR gene rearrangements, supplemented by detection of monoclonal proteins, is advantageous in monitoring NBS patients before severe complications of the disease, including cancer, appear.
机译:在57名奈梅亨断裂综合征患者中监测了选定的病毒和免疫参数,将其作为早期发现恶性肿瘤变化的建议工具。分析了以下参数:(1)病毒感染; (2)单克隆蛋白; (3)外周血淋巴细胞中的B细胞和T细胞受体基因重排。在以EBV为主的患者中,有68.4%的患者检测到病毒感染,其次是HBV(19.2%)和HCV(8.8%)。在38.6%的病例中检测到的单克隆丙种球蛋白病与EBV DNA(p = 0.002)和HCV RNA(p = 0.04)有关。 73.9%的患者发生了克隆性Ig和/或TCR基因重排。至少有一项研究参数的出现在22例患者发生恶性肿瘤之前。对于病毒感染和Ig / TCR基因重排的系统PCR分析,再加上单克隆蛋白的检测,对于在疾病的严重并发症(包括癌症)出现之前监测NBS患者具有优势。

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