儿童系统性EBV阳性T细胞淋巴组织增殖性疾病的研究进展  

Progress of childhood systemic Epstein-Barr virus positive T-cell lymphoproliferative disease

作　　者：徐海生 缪红军[1] XU Haisheng;MIAO Hongjun(Children's Hospital of Nanjing Medical University,Jiangsu Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院,江苏南京210008

出　　处：《现代肿瘤医学》2023年第10期1948-1951,共4页Journal of Modern Oncology

摘　　要：在2016年WHO淋巴瘤分类中,将儿童系统性EBV阳性T细胞淋巴组织增殖性疾病(childhood systemic Epstein-Barr virus positive T-cell lymphoproliferative disease, CSEBV^(+)T-LPD)更名为儿童系统性EBV^(+)T细胞淋巴瘤(systemic EBV^(+)T-cell lymphoma of childhood),以强调其爆发性的临床进程。CSEBV^(+)T-LPD缺乏特异性临床特征,确诊需要综合分析临床表现、病理形态学、免疫组化检测、EBV-EBER原位杂交和TCR基因检测。EBV在促进淋巴瘤发生中起着重要作用,但其确切机制尚不清楚,需要更加深入的研究,以便为做出准确诊断和实施可靠治疗提供依据。目前尚无CSEBV^(+)T-LPD治疗规范,预后差,病死率高,异基因造血干细胞移植为目前儿童CSEBV^(+)T-LPD有效治疗手段,异基因造血干细胞移植能有效清除EBV感染的细胞,并重建对EBV的正常免疫。In the latest classification of lymphoma by the World Health Organization in 2016,childhood systemic Epstein-Barr virus positive T-cell lymphoproliferative disease(CSEBV^(+)T-LPD)was renamed as"systemic EBV^(+)T-cell lymphoma of childhood"to emphasize its explosive clinical process.The disease lacks specific clinical features and the diagnosis requires comprehensive analysis of clinical manifestations,pathological morphology,immunohistochemical testing,EBV-EBER in situ hybridization and TCR gene testing.EBV plays an important role in promoting the occurrence of lymphoma,but its exact mechanism is still unclear,and more in-depth research is needed to provide a basis for accurate diagnosis and reliable treatment.At present,there is no standard treatment plan or consensus for the systemic EBV-positive T-cell lymphoid tissue proliferative disease in children.The overall prognosis of the disease is poor and the mortality rate is high.Allogeneic hematopoietic stem cell transplantation is currently an effective treatment for children with CSEBV^(+)T-LPD.Gene hematopoietic stem cell transplantation can effectively eliminate EBV-infected cells and rebuild normal immunity to EBV.

关 键 词：EB病毒 淋巴组织增殖性疾病 淋巴瘤 

分 类 号：R733.4[医药卫生—肿瘤]