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42例EBV阳性成熟T/NK细胞肿瘤的临床分析

Clinical analysis of 42 cases of EBV-positive mature T/NK-cell neoplasms.

作者信息

Yang Haiyan, Fu Gan, Liu Jia, Da Zhenzhen, Cheng Xiaoye, Chen Cong, Li Yan, Fu Bin, Li Xiaolin

机构信息

Department of Hematology, Xiangya Hospital of Central South University, Changsha, Hunan 410008, P.R. China.

出版信息

Exp Ther Med. 2017 Jul;14(1):567-574. doi: 10.3892/etm.2017.4531. Epub 2017 Jun 1.

Abstract

The pathogenesis of Epstein-Barr virus-positive (EBV) mature T-cell and natural killer (NK)-cell neoplasms is challenging to understand. The clinical features are diverse, with no typical manifestation. Therefore, it is important to analyze the association of the clinical characteristics and prognoses of patients with various factors associated with EBV T/NK-cell neoplasms, particularly extranodal NK/T cell lymphoma, systemic EBV T/NK cell lymphoproliferative disorders, aggressive NK cell leukemia and EBV peripheral T-cell lymphoma. Therefore, 42 cases of EBV T/NK-cell neoplasms with information on age, gender, fever, LDH level, complete blood count (CBC) and immunophenotype (CD5/CD20) were retrospectively analyzed to examine the clinical features, prognoses and related factors. It was found that patients ≤60 years old accounted for 86% of cases. The frequency of stage III/IV disease was higher in groups with pancytopenia (P=0.005), high LDH level (P=0.020), CD5-expression status (P=0.031) and fever (P=0.024). There were significant differences in the mean International Prognostic Index (IPI) scores according to the presence or absence of fever (P=0.022), elevated or normal lactose dehydrogenase (LDH) levels (P=0.001), and pancytopenia or normal complete blood count (CBC; P=0.046). Analysis of overall survival showed that CD5 expression, CBC, IPI scores and LDH levels were factors associated with OS. CD5 expression (P=0.003), CBC (P=0.003) and IPI scores (P=0.017) were identified to be important risk factors on the basis of Cox regression analysis. The mean survival time was longer in the CD5, CD20 and normal CBC groups, and there was no clear difference in survival time according to LDH level or fever. In summary, CD5 and CD20 may be prognostic factors in EBV T/NK lymphoid neoplasms, and CBC and fever are most likely to influence the IPI score and Ann Arbor stage.

摘要

爱泼斯坦-巴尔病毒(EBV)阳性的成熟T细胞和自然杀伤(NK)细胞肿瘤的发病机制难以理解。其临床特征多样,无典型表现。因此,分析EBV T/NK细胞肿瘤(特别是结外NK/T细胞淋巴瘤、系统性EBV T/NK细胞淋巴增殖性疾病、侵袭性NK细胞白血病和EBV外周T细胞淋巴瘤)患者的临床特征与预后和各种相关因素之间的关联非常重要。因此,回顾性分析了42例有年龄、性别、发热、乳酸脱氢酶(LDH)水平、全血细胞计数(CBC)和免疫表型(CD5/CD20)信息的EBV T/NK细胞肿瘤病例,以研究其临床特征、预后及相关因素。发现年龄≤60岁的患者占病例的86%。全血细胞减少组(P=0.005)、LDH水平高(P=0.020)、CD5表达状态(P=0.031)和发热组(P=0.024)中III/IV期疾病的发生率更高。根据是否发热(P=0.022)、乳酸脱氢酶(LDH)水平升高或正常(P=0.001)以及全血细胞减少或全血细胞计数(CBC)正常情况(P=0.046),平均国际预后指数(IPI)评分存在显著差异。总生存分析表明,CD5表达、CBC、IPI评分和LDH水平是与总生存相关的因素。基于Cox回归分析,CD5表达(P=0.003)、CBC(P=0.003)和IPI评分(P=0.017)被确定为重要的危险因素。CD5、CD20和CBC正常组的平均生存时间更长,根据LDH水平或发热情况,生存时间无明显差异。总之,CD5和CD20可能是EBV T/NK淋巴肿瘤的预后因素,CBC和发热最有可能影响IPI评分和Ann Arbor分期。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a175/5488386/0ab9e660a992/etm-14-01-0567-g00.jpg

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