摘要目的 分析结外鼻型NK/T细胞淋巴瘤(NKTL)的临床病理特点,探讨其与疾病预后的关系,为临床个体化治疗提供依据.方法 回顾117例确诊为NKTL患者的病理临床资料.病理诊断均采用常规组织学、免疫组织化学、T细胞重排和原位杂交方法检测EB病毒(EBV)编码小RNA(EBER).EBV DNA拷贝数检测采用PCR方法.所有患者经过国际预后指数(IPI)和Ki-67等指标临床评估后均采用化疗和放疗联合治疗.随访患者并对临床指标与2年总生存(0S)率和无进展生存(PFS)率的关系进行单因素分析.结果 免疫组织化学结果提示各指标阳性率:CD3为90.6％,CD56为94.0％,CD45RD为92.9％,TIA为97.9％,Granzyme B为97.7％,EBER为100.0％.117例患者中位年龄为43.2岁(14～77岁),原发鼻者95例(81.2％),Ki-67平均值为(48.3±2.6)％；原发非鼻者22例(18.8％),包括原发咽喉、舌、扁桃体、淋巴结、皮肤、肝、肠、中枢神经系统和睾丸.与原发鼻的NKTL患者相比,原发肝和肠者Ki-67值较高,且Ki-67值大于80％的NKTL患者均在1年内死亡.Ki-67值为60％～80％的45例2年OS率为60.0％,与Ki-67值为30％～60％的33例OS率(86.3％)和Ki-67值小于30％的8例OS率(100.0％)分别相比,差异均有统计学意义(P=0.047、0.011).Ki-67值为60％～80％者2年PFS率为36.0％,与Ki-67值为30％～60％者PFS率(57.5％)相比,差异无统计学意义(P=0.07),与Ki-67值小于30％者PFS率(78.0％)相比,差异有统计学意义(P=0.02).CD56阴性的8例CD3表达阳性,TCR重排均为阳性,提示为T细胞来源,原发部位均为鼻部,无全身症状,2年OS率和PFS率分别为100.0％和70.0％,高于CD56阳性患者,差异均有统计学意义(P=0.03、0.02).13例检测了EBV DNA拷贝数,5例高于正常值者OS率为60.0％,余8例OS率为100.0％.结论 除了IPI外,NKTL原发部位和病理特征,尤其是Ki-67、CD56、EBER和EBV DNA拷贝数与其临床预后的关系不容忽视,也是NKTL预后判断的独立因素.

abstractsObjective To improve the understanding of clinical and pathological features of extranodal NK/T cell lymphoma,nasal type (NKTL) with poor prognosis and provide experiential references via a retrospective analysis.Methods 117 NKTL cases in a single center were retrospectively analyzed of their pathologic diagnoses and clinical manifestations,especially primary sites.Pathological examinations were mainly depended on morphology,immunohistochemisty for immunophenotype,and in situ hybridization for Epstein-Barr virus (EBV) encoded small RNA (EBER).Polymerase chain reaction (PCR) for whole-blood EBV DNA and T-cell receptor (TCR) gene rearrangement was performed.Chemotherapy and radiotherapy were the main treatments.International prognostic index,Ki-67,2 years overall survival (OS) rate and progressive free survival (PFS) rate according to the clinical characteristics were included in the univariable analysis.Results The positive rate for CD3 was 90.6 ％,94.0 ％ for CD56,92.9 ％ for CD45RO,97.9 ％ for TIA,97.7 ％ for Granzyme B and 100.0 ％ for EBER,respectively.The median age was 43.2 (14-77) years old.The primary nasal NKTL was 95 cases (81.2 ％) and their average Ki-67 was (48.3±2.6) ％.Other primary extranodal NKTL was 22 cases (18.8 ％),including primary posterior pharyngeal wall,tongue,tonsil,laryngeal,lymph nodes,skin,liver,intestinal,central nervous system and testis.Patients with primary liver or intestinal NKTL or Ki-67 greater than 80 ％ died in the first year.Patients with primary liver and intestinal NKTL had higher Ki-67 than patients with primary nasal.Compared to the 2 years OS rate 60.0 ％ and PFS rate 36.0 ％ of patients with Ki-67 from 60 ％ to 80 ％,the OS rate (86.3 ％) and PFS rate (57.5 ％) of patients with Ki-67 from 30 ％ to 60 ％ were higher (P =0.047,0.070),and the OS rate (100.0 ％) and PFS rate (78.0 ％) of patients with Ki-67 less than 30 ％ were also higher (P =0.01,0.02).2 years OS rate of 8 CD56 negative patients whose T cell rearrangement was positive and primary sites were nasal was higher than that of 109 CD56 positive patients (P =0.03,0.02).Among the 13 EBV DNA samples detected,8 samples were normal and OS rate was 100.0 ％.5 samples had more than 6.1×107 copies/ml and OS rate was 60.0 ％.Conclusion It is implied that Ki-67,CD56,EBER,EBV-DNA and primary site are related with the prognosis of NKTL.