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目的:分析儿童EB病毒(Epstein-Barr virus，EBV)相关淋巴组织增殖性疾病的临床特点，提高对该病的认识。方法:对浙江大学医学院附属第一医院2014年1月至2018年12月收治的确诊EBV相关淋巴组织增殖性疾病患儿的临床资料以及治疗进行回顾性总结。根据疾病病程和严重程度分为轻症组和重症组。采用n χ2检验、秩和检验分析两组临床特征。n 结果:共收集到114例(男53例，女61例)病例资料，中位年龄6岁(范围0～17岁)，轻症组107例(93.86%)，其中EBV感染38例、传染性单核细胞增多症69例；重症组7例(6.14%)，以T细胞或NK细胞增殖为主(6例)。重症组反复发热时间明显较长，EBV-DNA的拷贝数较高，与轻症组比较，差异无统计学意义(n χ2＝0.957，n P>0.05)。重症组的IgM水平低于轻症组，秩和检验结果差异有统计学意义(n Z＝-2.041，n P0.05)。轻症组患儿经抗病毒等治疗后转归良好。重症组中1例确诊为种痘样水疱病样EBV相关淋巴组织增殖性疾病(HV-like LPD)，抗病毒治疗效果欠佳，经大剂量丙种球蛋白冲击和硼替佐米联合甲泼尼龙治疗后病情好转；1例失访；1例EBV相关噬血细胞综合征化疗后死亡，另1例EBV相关噬血细胞综合征和1例EBV相关淋巴瘤在移植后死亡。n 结论:EBV相关淋巴组织增殖性疾病常表现为反复发作“传染性单核细胞增多症”样症状。EBV感染伴有免疫功能紊乱如免疫球蛋白IgM、IgA降低，高DNA拷贝数和高IgE，或所感染的淋巴细胞类型为NK或T细胞的病例可能进展为重症EBV相关淋巴组织增殖性疾病，应提高警惕。移植和化疗对重症EBV相关淋巴组织增殖性疾病可能有效，但仍不确切。“,”Objective:To investigate the clinical features of Epstein-Barr virus associated lymphoproliferative disease in children and to improve the understanding of this disease.Methods:This study included the children with Epstein-Barr virus associated lymphoproliferative disease admitted to the First Affiliated Hospital of College of Medicine of Zhejiang University from January 2014 to December 2018.Data of these children were collected, including age, clinical manifestations, laboratory results, treatment and outcome.The clinical features and therapeutic effects were analyzed.Results:A total of 114 cases(mean age 6 years, 0~17 years)were enrolled in this study, including 53 males and 61 females.There were 107 cases(93.86%) in the mild group (38 cases of EBV infection and 69 cases of infectious mononucleosis) and 7 cases in the severe group (6.14%). Six cases of the severe group were T cell or NK cell proliferation.Compared with the mild group, the load of EBV-DNA was higher in the severe group, but there was no significant difference(n χ2=0.957, n P>0.05). The IgM in severe group was significantly lower(n Z=-2.041, n P<0.05). But the differences in the level of immune function including IgA, IgG, CD4n + cell and CD8n + cell between the severe group and the mild group were not significant.The cases in the mild group had improved after antiviral treatments.Among the severe group, 3 cases survived after treatment, another 1 case was diagnosed as hydroa vacciniforme-like EBV-related proliferative disease (HV-like LPD). After antiviral treatment, the effect was not good, then after high-dose IVIG treatment and Bortezomib combined with methylprednisolone treatment, the EBV-DNA load decreased and the condition improved.While 1 case lost to follow-up, there were 2 cases with EBV-associated hemophagocytic syndrome and 1 case with EBV-associated lymphoma died after chemotherapy or transplantation.n Conclusion:EBV-associated lymphoproliferative disease may manifest as a condition similar to infectious mononucleosis.High IgE, low IgM or high DNA load may indicate poor prognosis.Immune function after EBV infection may have different effects on prognosis.When the infected lymphocyte types are NK or T cells, it may indicate poor prognosis.The efficacy of transplantation and chemotherapy in severe cases is still uncertain.