中文摘要：

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?

英文摘要：

Objective 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 Siehuan 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 / lI. 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,