结外NK/T细胞淋巴瘤治疗的进展

摘要

Extranodal NK/T-cell lymphoma is a rare pathological type, incidence of which is 2％ - 10％ of the primary NHL cases, showing a broad morphologic spectrum with frequent necrosis.Extranodal NK/T-cell lymphoma occurs more frequently in Asian population, especially in Southern China and Southeast Asian population.It is reported that the incidence of ENKL among lymphomas ( 1314 cases) in Asian countries was 4 times as much as the Western countries (22％ vs.5％ ) by the International Peripheral T-cell Lymphoma Project.Nasal NK/T-cell lymphoma most commonly occurred in the nasal cavity or other parts of the upper aerodigestive tract, and is highly associated with EBV (Epstein-Bart virus) infection, which is highly aggressive and its prognosis generally is poor.The mean survival time is about 12 -38 months.It is important to accrately assess the patients prognosis for an optimal treatment.Localized disease (stage Ⅰ and Ⅱ) often has a relatively good prognosis through local radiation or combined therapy.There is a paucity of data to guide therapy in advanced disease.Generally, combining therapy is the most commonly selected approach for advanced disease.The conditions of the patients with advanced, relapsed or refractory diseases have been improved by high-intensity chemotherapy combined with radiation, and a regimen containing L-asparaginase L-Asp.Recently some studies have demonstrated promising outcomes in the selected cases by high-dose chemotherapy supplemented with auto- or allo-HSCT.Targeting therapy is also developing quickly.This current review mainly focuses on the advance of treatment for extranodal NK/T-cell lymphoma.%结外NK/T细胞琳巴瘤(extranodal NK/T-cell lymphoma,ENKL)属于一种罕见的病理类型,它常常表达广泛的形态学表型、出现坏死,其发生率约占原发性非霍奇金淋巴瘤(NHL)的2%-10%.ENKL多发于亚洲人群,特别是中国的南部及东南亚人群.据国际外周T细胞淋巴瘤计划统计,在1 314名琳巴瘤患者中亚洲国家较西方国家ENKL发生率高出4倍.ENKL最常发生在鼻腔或上呼吸消化道,常常伴随EB病毒感染,具有高侵袭性、预后差等特点,中位总生存时间约12-38个月.进行准确的分期评估对选择最佳治疗方案非常重要,局灶性疾病(Ⅰ期和Ⅱ期)通过局部放疗或联合化疗常常能够获得相对好的预后.由于病例相对缺乏,对于晚期、复发、难治疾病尚无标准的治疗方案,在目前综合治疗是最常用的方法,如高强度的化疗联合放疗及L-门冬酰胺酶的使用均可获得较好的结局.近年来有文献报道,大剂量化疗联合造血干细胞移植亦可以作为治疗复发难治晚期疾病的一种补救方法,而且有一定的前景,靶向治疗亦有所发展.本文综述了NK/T细胞淋巴瘤治疗的研究进展.