CAJ | 학술논문

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.
배경여목적:신강작위아국다민족취거지지일,근년래악성림파류발병솔정축년상승추세.유연구현시유오이족림파류적발병솔이거상견악성종류적제5위,차중위발병년령야정현년경화추세.위료해신강지구비형결외NK/T세포림파류적발병솔급충족、유전혹배경인소시부여종류적발생상관,본연구통과관찰63례신강다민족환자비형결외NK/T세포림파류림상、병리특정、면역표형이급여EB병독적상관성,제고대차류종류적조기진단화감별진단능력.방법:회고성분석2006년3월—2012년3월재본원취진적63례ENKTCL림상자료급예후,광경하관찰종류조직형태학특정,면역조직화학EnVision이보법분석종류적면역표형CD3、CD56、CD2、CD5、CD8、CD4급세포독소상관단백:TIA1、립매B、천공소적양성표체솔;원위잡교방법검측종류조직중EBER적표체.결과:환자이남성、40~50세년령조거다,한족적발병솔고우소수민족.54례발생우비강등상호흡소화도부위,기여위비외결외병변.소유병례조직학표현,균유불동정도적배사,병가견정도불등적염증반응.종류세포체적대소불등차다유이형표현,병출현“혈관중심성”화“혈관파배성”생장모식.소유종류병례불동정도지표체T세포표지물,53례(84%)표체CD56.소유병례균지소표체1충세포독소상관단백분자.57례경검병례EBER전부양성표체.결론:신강지구비형결외NK/T세포림파류이한족중년남성위다견,다발생우비강등처,존재고수평EB병독감염.림상조기진단상수결합병리형태관찰、면역표형분석급EB병독검측.
Background and purpose:Xinjiang, a multi-ethnic region in China, showed an increasing incidence rate of extranodal NK/T cell lymphoma, nasal type (ENKTCL) in recent years. Studies have shown that the Uyghur lymphoma incidence has increased to become the fifth most common tumor type, and the median age of onset has been decreasing. This study investigated the incidence and ethnic distribution in Xinjiang of the nasal type extranodal NK/T cell lymphoma and variation in racial, genetic, or environmental factors if it was associated with its incidence. Sixty-three cases of extranodal NK/T cell lymphoma, nasal type (ENKTCL) occurring in the multiple nationalities in Xinjiang were studied to see the clinical and pathologic features of this type of tumor and its relationship to Epstein-Barr virus (EBV), with an emphasis on the diagnostic approach to those problematic cases. Methods:Sixty-three patients with ENKTCL were treated in our hospital from Mar. 2006 to Mar. 2012. All cases were submitted for histologic assessment, the immunostaining for LCA, CD3, CD56, CD2, CD5, CD8, CD4 and cytotoxic associated protein, such as TIA1, granzyme B, perforin was performed using EnVision, and in situ hybridization detection for the EBER, clinical information were also analyzed. Results:Male patients, aged between 40 to 50 years, predominated the present series, with Han nationalities overtly out-numbered Uyghur patients. Fifty-four cases arose from the nasal cavity or the other upper respirodigestive tract whereas the remaining ones involving other extranodal sites. All the cases exhibited similar histologic changes: Necrosis and inflammatory infiltration were frequently present. Cytologic atypia as well as the angiocentric and angiodestructive growth pattern of the tumor cells were consistently noted, although the size and contour of the tumor cells varied considerably in different cases. All the cases expressed one or several T-cell markers and at least one of the cytotoxic molecules. Fifty-three cases (84%) expressed CD56, 57 cases submitted for EBER detection showed positive results. Conclusion:ENKTCL affects predominantly median-aged Han Chinese in Xinjiang, with a predilection of involving nasal cavity and a strong association with high load of EBV infection. Early and correct identification of the disease in daily practice depend on the combination of morphologic evaluation, immunophenotypic assessment, and detection for EBER.