鼻型结外NK/T细胞淋巴瘤的诊断与治疗进展

Advances in diagnosis and treatment of extranodal NK/T-cell lymphoma nasal-type

鼻型结外NK/T细胞淋巴瘤以往被称为致死性中线肉芽肿、中线恶性组织细胞癌。新近WHO淋巴组织肿瘤分类和REAL分类认为本病是非霍奇金淋巴瘤 (NHL)的一个独立病种。其瘤细胞大部分来源于外周NK细胞 ,少部分来自NK样 (细胞毒性 )T细胞。鼻型NK/T细胞淋巴瘤少见 ,多分布于亚洲及南美地区。发病与EB病毒密切相关。病理组织学、免疫表型特征结合临床发病部位是诊断的主要依据。本病多预后不良。传统疗法及近年来尝试化疗 (CHOP类方案 )联合病灶野照射的综合治疗 ,疗效均不佳。新近报告应用左旋门冬酰胺酶为主的挽救化疗方案联合病灶野放疗 ,疗效有所提高 ;自体或同种异基因造血干细胞移植治疗的研究正在进行。

Extranodal NK/T-cell lymphoma,nasal-type previously known as lethal midline granuloma,midline reticulosis,is recognized as a distinct clinical pathological entity of non-Hodgkin's lymphoma,expressing NK-cell or NK-like cytotoxic T-cell origin in the new WHO classification of lymphoid neoplasms and the Revised European-American lymphoma(REAL)classification. It comprises approximately 3%～8.7% of non-Hodgkin's lymphoma in Asia,South and Central America,showing a racial predisposition for people of Asian,Latin,and South American descent.In Europe and America,it accounts for less than 1% of the lymphoma.It si highly associated with EBV,providing support for the role of EBV in tumor pathogenesis.Histological assessment shows pleomorphic cell infiltration(i.e small,medium-size or large atypical lymphoid cells).marked coagulative necrosis and often along with angioinvasion.This entity often primaily presents with midfacial progressively destructive lesion.Extranodal NK/T-cell lymphoma nasal-type is conventionally treated with radiation therapy or radiation.therapy combined with CHOP-like chemotherapy.However,orognosis is poor for most patients.There is no consensus on the optimal therapeutic regimen,The 5-year overall survial rate is 30%～44% About 60% of the patients are clinically aggressive and frequently refractory to conventional CHOP regimen. Recently,L-asparaginase based regimen as a salvage regimen was reported to treat the oatients with refractory nidline NK/T-cell lymphoma.L-asparaginsase based salvage regimen improved efficacy,and the 5-year overall survival rates was 50% for CHOP failures,suggesting a promising new salvage regimen.High-dose caemtherapy with stem rescue has been used but the experience has been limited and unconfirmed.Clinical stage,age,PS,and B,symptom were reported to have prognostic significance in NK/T-cell lymphoma nasal type.Among them,clinical stage was the most frequently cited prognostic factor to all the patients.Because of the rarity of extranodal NK/T-cell lymphoma,nasal-type,muticenter coopertaion is nocessary to improve the treatment outcome and prognosis.