EB病毒阳性淋巴组织增殖性疾病13例临床与病理特征  

Clinical and pathological features of 13 children with Epstein-Barr virus-positive lymphoproliferative diseas

作　　者：陈天明[1] 邓志娟[2] 胡冰[1] 胡惠丽[1] 陈荷英[1] 李绍英[1] 刘钢[1] Chen Tianming;Deng Zhijuan;Hu Bing;Hu Huili;Chen Heying;Li Shaoying;Liu Gang(Department of Infectious Disease,Beijing Children＇s Hospital Affiliated to Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院感染内科,100045 [2]首都医科大学附属北京儿童医院病理科,100045

出　　处：《中华儿科杂志》2018年第10期759-764,共6页Chinese Journal of Pediatrics

摘　　要：的探讨儿童EB病毒阳性淋巴组织增殖性疾病（EBV＋LPD）的临床表现、病毒学特征、组织病理学特点和临床转归。方法对北京儿童医院感染内科2011年1月至2016年12月收治的13例组织病理学确诊的儿童EBV＋LPD的临床资料及随访情况进行回顾性病例总结。结果13例患儿中，男5例、女8例。中位年龄6.0岁（范围1.3-15.0岁）。从发病到诊断的中位时间为3个月（1-24个月）。13例均有发热，9例肝脾淋巴结肿大，4例仅有淋巴结肿大；7例外周血细胞降低，7例肺受累，3例中枢神经系统受累，3例心脏受累，3例肠道受累，2例皮肤受累，腹部包块1例。13例均行全血EB病毒-DNA检查，拷贝数1×108/L-1×1011/L。病理学检查：6例为淋巴结病理，2例为皮肤病理，肺、回肠黏膜、肝脏、腹腔肿物、骨髓组织病理各1例；13例中9例为系统性EB病毒阳性T细胞淋巴组织增殖性疾病（EBV＋T-LPD），2例为种痘样水疱病（HV），2例为EB病毒阳性弥漫性大B细胞淋巴瘤（EBV＋ DLBCL）。对13例患儿分别随访至出院后2 d-65个月，9例系统性EBV＋T-LPD，其中1例短期内死亡，1例进展为T细胞淋巴瘤死亡，2例行造血干细胞移植痊愈，1例行噬血细胞综合征2004方案化疗后痊愈，4例病情尚稳定；2例HV患儿1例发展为种HV样淋巴瘤死亡，另外1例仍有症状；2例EBV＋ DLBCL，1例出院后短期死亡，1例化疗后病情稳定。结论儿童EBV＋LPD常见表现为慢性反复发热、淋巴结、肝脾肿大，另外可累及肺、中枢神经系统、肠道、皮肤等脏器；对于慢性反复不明原因发热伴有淋巴结和（或）肝脾肿大的，伴全血EB病毒-DNA载量持续显著增高时，应警惕EBV＋LPD，尽早行增殖病灶组织活检明确诊断；本病预后差，部分进展为淋巴瘤，造血干细胞移植治疗有效。ObjectiveTo summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease （EBV＋LPD） in children.MethodsThe clinical and follow-up data of 13 children histopathologically diagnosed as EBV＋LPD in the Department of Infectious Disease of Beijing Children＇s Hospital between January 2011 and December 2016 were summarized.ResultsOf the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years （range 1.3 to 15.0 years）. The median duration between disease onset and diagnosis was 3 months （range 1 to 24 months）. All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×108/L to 1×1011/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease（EBV＋ T-LPD）, 2 cases with hydroa vacciniforme （HV） and 2 cases with EBV-positive diffuse large B-cell lymphoma （EBV＋ DLBCL） . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV＋T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis（HLH） 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still

关 键 词：疱疹病毒4型 人 淋巴组织增殖性疾病 儿童 

分 类 号：R725.1[医药卫生—儿科]