摘要
结外NK/T淋巴细胞淋巴瘤鼻型(ENKTL)为起源于NK或细胞毒T淋巴细胞的一种高度侵袭性非霍奇金淋巴瘤(NHL),发病与EB病毒(EBV)感染密切相关,东南亚和中南美洲发病率高。本病好发于鼻腔、鼻咽、鼻窦、腭部等呼吸消化道上段中线部位,肿瘤细胞弥散性浸润,呈血管中心性破坏性生长,引起进行性毁损性坏死为本病显著临床病理特征。近10余年来国际上深化了ENKTL分子遗传发病机制的认识,完善和优化了分期和危险度分组系统,通过局部放疗、同步放化疗和三明治疗法等治疗策略,显著改善Ⅰ、Ⅱ期肿瘤疗效和预后,但进展期肿瘤总体预后有待提高。新型免疫治疗有望成为复发难治性ENKTL的重要治疗手段,现将相关研究进展进行阐述。
Extranodal natural killer(NK)/T cell lymphoma, nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma, closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis, and is prevalent in East Asia and South America.Clinicopathologically, ENKTL is characterized by diffuse tumor cell infiltration, angiocentral destructive growth pattern, and prominent tissue necrosis.Typically, ENKTL presents with extranodal involvements, particularly the upper aerodigestive tract, such as nasal cavity, nasopharynx, paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis, refined tumor staging and risk assignment, and treatment strategies including local radiotherapy, concurrent chemoradiotherapy and sandwich therapy.Accordingly, response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors, though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.
作者
郭霞
高举
Guo Xia;Gao Ju(Department of Pediatrics,West China Second University Hospital,Sichuan University,Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) ,Ministry of Education,Chengdu 610041,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2019年第15期1136-1140,共5页
Chinese Journal of Applied Clinical Pediatrics
关键词
结外NK/T淋巴细胞淋巴瘤
鼻型
诊断
治疗
进展
Extranoda natural killer/T cell lymphoma, nasal type
Diagnosis
Treatment
Advances
