伴噬血细胞综合征EB病毒阳性T细胞淋巴瘤患者临床特征及疗效分析  

Clinical features and therapeutic efficacy analysis of Epstein-Barr virus-positive T-cell lymphoma patients with hemophagocytic syndrome

作　　者：徐嘉良 郑润辉[1] 罗晓丹[1] 覃鹏飞[1] 林竟韧 郜靓 谭获[1] 王春燕[1] Xu Jialiang;Zheng Runhui;Luo Xiaodan;Qin Pengfei;Lin Jingren;Gao Liang;Tan Huo;Wang Chunyan(Department of Hematology,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510260,China)

机构地区：[1]广州医科大学附属第一医院血液内科,广州510260

出　　处：《白血病．淋巴瘤》2021年第11期658-664,共7页Journal of Leukemia & Lymphoma

摘　　要：目的探讨噬血细胞综合征[又称噬血细胞性淋巴组织细胞增多症(HLH)]对EB病毒(EBV)阳性T细胞淋巴瘤(TCL)患者临床特征及治疗效果的影响。方法回顾性分析2015年11月至2020年8月于广州医科大学附属第一医院经病理检查确诊的23例EBV-TCL患者临床资料。按发病时是否伴HLH分为HLH组(10例)和非HLH组(13例),比较两组患者临床特征及预后。比较不同治疗方式、血浆EBV-DNA定量患者的疗效。结果23例患者中,Ann Arbor分期Ⅰ~Ⅱ期3例(13.0%),Ⅲ~Ⅳ期20例(87.0%);国际预后指数(IPI)评分1分3例(13.0%),2分4例(17.4%),3分8例(34.8%),4分8例(34.8%)。HLH组中侵袭性NK细胞白血病2例,儿童系统性EBV-TCL 3例,非HLH组无这两种病理类型患者。HLH组中发热、骨髓侵犯、IPI评分>2分、EBV-DNA>104拷贝/ml患者均多于非HLH组(均P<0.05)。所有患者化疗后客观缓解(完全缓解+部分缓解)率为47.8%(11/23);HLH组和非HLH组均有3例行造血干细胞移植,且均获得客观缓解;HLH组和非HLH组未行造血干细胞移植的7例和10例患者经过淋巴瘤方案化疗后,客观缓解分别为0例和5例,差异有统计学意义(P=0.044)。单纯化疗组17例患者中客观缓解5例,化疗+移植组6例患者均客观缓解,差异有统计学意义(P=0.039)。血浆EBV-DNA定量转阴的16例患者中客观缓解11例,持续阳性的7例患者均未客观缓解,差异有统计学意义(P=0.001)。所有患者1年总生存率为69.3%,2年总生存率为52.0%。HLH组中7例单纯化疗患者和3例化疗+移植患者的1、2年总生存率均分别为42.9%和66.7%。非HLH组中10例单纯化疗患者和3例化疗+移植患者的1年总生存率分别为80.0%和100.0%,2年总生存率分别为26.7%和100.0%。两组中化疗+移植患者的总生存均优于单纯化疗患者,差异均有统计学意义(均P<0.05)。结论EBV-TCL患者总体临床分期较晚,伴HLH的患者预后更差,治疗效果可能与血浆EBV-DNA定量相关。造血干细胞移植能提高�Objective To investigate the effects of hemophagocytic syndrome also known as hemophagocytic lymphohistiocytosis(HLH)on the clinical features and therapeutic efficacy of patients with Epstein-Barr virus-positive T-cell lymphoma(EBV-TCL).Methods The clinical data of patients with EBV-TCL diagnosed by pathological examination in the First Affiliated Hospital of Guangzhou Medical University from November 2015 to August 2020 were retrospectively analyzed.According to whether they were accompanied with HLH at the time of onset,patients were divided into HLH group(10 cases)and non-HLH group(13 cases),and the clinical features and prognosis of the two groups were compared.The curative effects of different treatment methods and patients with different plasma EBV-DNA titers were compared.Results Among 23 patients,3 cases(13.0%)were in Ann Arbor stage Ⅰ-Ⅱ,20 cases(87.0%)were in stage Ⅲ-Ⅳ;the International Prognostic Index(IPI)score was 1 point in 3 cases(13.0%),2 points in 4 cases(17.4%),3 points in 8 cases(34.8%),4 points in 8 cases(34.8%).In the HLH group,there were 2 cases of aggressive NK-cell leukemia and 3 cases of childhood systemic EBV-TCL.There were no cases of above two pathological types in the non-HLH group.In the HLH group,the proportions of patients with fever,bone marrow invasion,IPI score>2 points,and EBV-DNA>104 copies/ml were higher than those in the non-HLH group(all P<0.05).The objective response rate(complete remission plus partial remission)of all patients after chemotherapy was 47.8%(11/23);there were 3 cases undergoing hematopoietic stem cell transplantation in both the HLH group and the non-HLH group,and all achieved objective remission.The objective remission of 7 patients and 10 patients who did not undergo hematopoietic stem cell transplantation in the HLH group and non-HLH group after lymphoma chemotherapy had 0 case and 5 cases,respectively,and the difference was statistically significant(P=0.044).In the chemotherapy alone group,5 of 17 patients had objective remission,6 patients in th

关 键 词：淋巴瘤 T细胞 疱疹病毒4型 人 淋巴组织细胞增多症 嗜血细胞性 造血干细胞移植 

分 类 号：R733.1[医药卫生—肿瘤]