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背景与目的:新疆作为我国多民族聚居地之一,近年来恶性淋巴瘤发病率呈逐年上升趋势。有研究显示维吾尔族淋巴瘤的发病率已居常见恶性肿瘤的第5位,且中位发病年龄也呈现年轻化趋势。为了解新疆地区鼻型结外NK/T细胞淋巴瘤的发病率及种族、遗传或环境因素是否与肿瘤的发生相关,本研究通过观察63例新疆多民族患者鼻型结外NK/T细胞淋巴瘤临床、病理特征、免疫表型以及与EB病毒的相关性,提高对此类肿瘤的早期诊断和鉴别诊断能力。方法:回顾性分析2006年3月—2012年3月在本院就诊的63例ENKTCL临床资料及预后,光镜下观察肿瘤组织形态学特征,免疫组织化学EnVision二步法分析肿瘤的免疫表型CD3、CD56、CD2、CD5、CD8、CD4及细胞毒素相关蛋白:TIA1、粒酶B、穿孔素的阳性表达率;原位杂交方法检测肿瘤组织中EBER的表达。结果:患者以男性、40～50岁年龄组居多,汉族的发病率高于少数民族。54例发生于鼻腔等上呼吸消化道部位,其余为鼻外结外病变。所有病例组织学表现,均有不同程度的坏死,并可见程度不等的炎症反应。肿瘤细胞体积大小不等且多有异型表现,并出现“血管中心性”和“血管破坏性”生长模式。所有肿瘤病例不同程度地表达T细胞标志物,53例(84%)表达CD56。所有病例均至少表达1种细胞毒素相关蛋白分子。57例经检病例EBER全部阳性表达。结论:新疆地区鼻型结外NK/T细胞淋巴瘤以汉族中年男性为多见,多发生于鼻腔等处,存在高水平EB病毒感染。临床早期诊断常需结合病理形态观察、免疫表型分析及EB病毒检测。
Background and Objective: As one of the multi-ethnic settlements in China, Xinjiang’s morbidity rate of malignant lymphoma has been increasing year by year. Studies have shown that the incidence of Uygur lymphoma has become the fifth most common malignant tumor, and the median age of onset also showed a trend of younger. To understand the incidence of nasal type NK / T cell lymphoma in Xinjiang and whether race, genetic or environmental factors are related to tumorigenesis, this study was to observe the nasal type extranodal NK / T cell lymphoma Tumor clinical, pathological features, immunophenotype and Epstein-Barr virus and improve the early diagnosis and differential diagnosis of such tumors. Methods: The clinical data and prognosis of 63 cases of ENKTCL treated in our hospital from March 2006 to March 2012 were retrospectively analyzed. The morphological features of tumor were observed under light microscope. The immunohistochemical EnVision two-step method was used to analyze the immunophenotype The positive expression rates of CD3, CD56, CD2, CD5, CD8, CD4 and cytotoxin related proteins TIA1, granzyme B and perforin were detected by immunohistochemistry. The expression of EBER in tumor tissue was detected by in situ hybridization. Results: The majority of patients were male, 40- to 50-year-old age group, the incidence of Han was higher than that of ethnic minorities. 54 cases occurred in the upper respiratory tract, nasal cavity and other parts of the extranasal extranodal disease. All cases of histological manifestations, have varying degrees of necrosis, and visible varying degrees of inflammatory response. Tumor cells vary in size and are often characterized by heterotrophic appearance, with “vascular center” and “vascular destructive” growth patterns. All tumor cases expressed T cell markers to varying degrees, and 53 (84%) expressed CD56. All cases expressed at least one cytotoxic-related protein. All cases of EBER were positive in 57 cases. Conclusion: Nasal type extranodal NK / T cell lymphoma in Xinjiang is more common in middle-aged Han men and occurs mostly in the nasal cavity with high level of Epstein-Barr virus infection. Early clinical diagnosis often combined with pathological observation, immunophenotyping and Epstein-Barr virus detection.