1例EBV阴性的结外鼻型NK/T细胞淋巴瘤患者的临床分析  

Clinical Analysis of EBV-Extranodal NK/T Cell Lymphoma Patients

作　　者：李慧敏 常香香 黄来全 LI Hui-Min;CHANG Xiang-Xiang;HUANG Lai-Quan(Graduate School of Wannan Medical College,Wuhu 241002,Anhui Province,China;Department of Hematology,Yijishan Hospital of Wannan Medical College,Wuhu 241001,Anhui Province,China)

机构地区：[1]皖南医学院研究生学院,安徽芜湖241002 [2]皖南医学院弋矶山医院血液内科,安徽芜湖241001

出　　处：《中国实验血液学杂志》2021年第4期1156-1162,共7页Journal of Experimental Hematology

基　　金：安徽省教育厅高校自然科学研究重点项目(KJ2017A263);安徽省自然科学基金资助项目(1708085MH231);皖南医学院质量工程项(2017jyxm05);安徽省省级教育质量工程项目(2018jyxm1268)。

摘　　要：目的:探讨1例EBV阴性的结外鼻型NK/T细胞淋巴瘤(ENKTL)患者的临床特点和诊治方法。方法:对1例EBV阴性的ENKTL患者的临床表现、诊断过程及治疗进行回顾性分析。结果:46岁女性,2016年9月在外院确诊为ENKTL(EBER^(+)),予以6个疗程CHOEP方案化疗及鼻咽部局部放射治疗,未定期随访复查。2020年3月20日患者因全身多处出现皮疹,右膝关节处破溃2月余于本院就诊。组织病理检查结果显示,炎性渗出及坏死组织,其内见异型淋巴细胞,部分围绕血管生长;免疫组化示:异型淋巴细胞CD3(部分^(+))、CD43^(+)、CD20^(-)、PAX-5^(-)、CD4^(-)、CD8^(-)、CD56^(+)、GrB^(+)、TIA-1^(+)、Ki-67(10%^(+)),显色原位杂交(CISH):EBER^(-)。结合PET-CT、骨髓细胞形态学、流式细胞学、染色体核型分析、TCR重排等检查,诊断为NK/T细胞淋巴瘤复发(鼻型)IV期B组(CA分期)、NK/T细胞淋巴瘤皮肤累及。2020年4月4日给予CEOP-L方案化疗4个疗程,并给予中枢淋巴瘤预防性鞘内化疗,复查PET-CT达到PR,调整为Gemox-L方案治疗中。结论:EBV-的ENKTL临床罕见,容易误诊,应注意与外周T细胞淋巴瘤鉴别;本例患者的治疗参照EBV阳性ENKTL方案,短期疗效佳。Objective: To investigate the clinical characteristics, diagnosis and treatment of 1 case EBV negative extranodal NK/T cell lymphoma(ENKTL) patients. Methods: The clinical manifestations, diagnosis and treatment of one case ENKTL patients with EBV negative were analyzed retrospectively. Results: A 46-year-old woman diagnosed as positive for exosanal NK/T cell lymphoma(EBER^(+)) in September 2016 was treated by 6 courses of CHOEP regimen chemotherapy and local nasopharyngeal radiotherapy, without regular follow-up review. On March 20, 2020, the patient was admitted to our hospital for multiple rashes and rupture of the right knee joint for morethan 2 months. Histopathology showed inflammatory exudation and necrotic tissue, atypia lymphocytes were observed, and part of them were grew around the blood vessels. Immunohistochemistry showed: heterotypic lymphocytes CD3(partial ^(+)), CD43^(+), CD20^(-),PAX-5^(-), CD8^(-), CD8^(-), CD56^(+), GrB^(+), TIA-1^(+), Ki-67(10%^(+)), and chromogenic in situ hybridization(CISH): EBER^(-), the patient was diagnosed as recurrent NK/T cell lymphoma(nasal type) stage IV B group(CA stage) and skin involvement of NK/T cell lymphoma by combining PET-CT, bone marrow cell morphology, flow cytometry, chromosome karyotype analysis and TCR rearrangement, etc. On April 4, 2020, the patient was treated by CEOP-L regimen(4 courses) and central lymphoma prophylactic intrathecal chemotherapy. PET-CT re-examination showed the patient achieved PR, and the treatment regimen was changed to Gemos-L regimen. Conclusion: EBV negative ENKTL is rare in clinic and easy to be misdiagnosed, so it should be distinguished from peripheral T cell lymphoma. This case was treated with EBV positive ENKTL regimen, with good short-term efficacy.

关 键 词：结外鼻型NK/T细胞淋巴瘤 EBER阴性 诊断 治疗 预后 

分 类 号：R733.1[医药卫生—肿瘤] R739[医药卫生—临床医学]