摘要目的 分析结外鼻型自然杀伤(NK)细胞/T细胞淋巴瘤(ENKTCL-N)临床病理特点,探讨NK细胞表面受体在ENKTCL-N中的表达及其与ENKTCL-N预后的关系,并建立预后模型.方法 对126例ENKTCL-N进行临床资料收集、组织病理学观察及随访,免疫组织化学EnVision或EliVision法检测CD16、细胞间黏附分子(ICAM)-1和淋巴细胞功能相关抗原(LFA)-1的表达,通过逆转录聚合酶链反应(RT-PCR)技术检测CD94、NKG2和KIR受体家族的表达,统计分析临床特征和组织病理学特征,以及上述标记的表达与患者预后之间的关系.结果 患者年龄6 ～ 84岁,中位年龄41岁,男女比为3.2:1.病变部位以鼻部(101例)多见,鼻外(25例)累及部位主要是消化道和皮肤,仅6例累及2个或以上结外部位,86.5％ (109/126)的患者就诊时处于临床Ⅰ/Ⅱ期.肿瘤细胞以中等大小细胞型多见,大细胞型者占9.5％ (12/126).CD56、CD16、CD94、LFA-1和ICAM-1的表达率分别为82.6％ (95/115)、15.1％ (19/126)、55.4％ (41/74)、40.5％ (51/126)和0,NKG2受体总体表达率为90.5％(67/74),NKG2受体可单独表达于肿瘤细胞表面.KIR受体家族总体表达率为33.8％(25/74),检出 KIR受体的病例中有20.8％(5/24)未出现限制性表达现象.126例患者平均生存时间20.2个月,中位生存时间15个月.对其临床表现、组织病理学形态和细胞表面抗原等方面的多种因素进行了比较,发现包括性别、年龄、部位、累及结外2个或以上部位、临床分期、CD16表达、CD94表达和LFA-1表达在内的8个因素与预后相关,其中年龄、累及部位、临床分期、CD16表达情况为独立预后因素.结论 根据年龄、累及部位、临床分期、CD16表达情况4个因素构建预后模型可以较为准确地评估ENKTCL-N的预后.鼻NK/T细胞淋巴瘤和鼻外NK/T细胞淋巴瘤在临床特点、预后和NK细胞表面抗原的表达方面存在明显差别.

abstractsObjective To analyze the clinicopathologic features of extranodal NK/T cell lymphoma,nasal type(ENKTCL-N),to explore the expression of NK cell-associated receptors in ENKTCL-N and the relationship with prognosis,and to establish a prognostic model.Methods One hundred and twenty-six cases of ENKTCL-N were selected from the files of the Department of Pathology,West China Hospital of Sichuan University.The relevant clinical and follow-up data were collected,and the histopathology was reviewed.All specimens were stained immunohistochemically for CD16,ICAM-1 and LFA-1.RT-PCR was used to detect the expression of CD94,NKG2 and KIR.The relationship between the prognosis of ENKTCL-N,clinical features,histopathological characteristics and expression of these markers were also analyzed.Results ENKTCL-N mainly occurred in middle-age and young patients (median age,41 years).The male to female ratio was 3.2∶ 1.Sites more commonly involved were the nose and upper aerodigestive tract whereas those for the non-nasal type were the skin and gut.Only six cases involved two or more extranodal sites.Most (86.5％,109/126) of the patients were in clinical stages Ⅰ /Ⅱ.The tumors showed predominately medium-sized tumor cells and large-sized tumor cells accounted for only 9.5％ (12/126).Coagulative necrosis was present in all cases.The expression rates of CD56,CD16,CD94,LFA-1 and ICAM-1 were 82.6％ (95/115),15.1％ (19/126),55.4％ (41/74),40.5％ (51/126) and 0,respectively.The expression rate of NKG2 receptor was 90.5％ (67/74) overall.NKG2 receptor expression was independent of CD94.The overall expression rate of KIR receptor was 33.8％ (25/74) and KIR receptor restriction was not detected in 20.8％ (5/24) of the cases.Follow-up data was available in all patients,with median and average survival time being 15 months and 20.2 months,respectively.Survival analysis showed that prognostic factors included the gender,age,disease type,extranodal involvement,stage,the expression of CD16,LFA-1 and CD94.Cox's proportional hazard regression analysis revealed four factors,age,involved site,stage and CD16 expression,were independent prognostic factors.Conclusions The age,disease type,stage and CD16 expression are independent prognostic factors.Establishment of a prognostic model based on the above four factors can be more accurate in the prognostication of ENKTCL-N.The differences in the clinical features,prognosis,and expression of NK cell-associated receptors are obvious between nasal NK-cell lymphoma and non-nasal NK-cell lymphoma.