摘要目的 探讨结外鼻型NK/T细胞淋巴瘤(EN-NK/TCL)的临床病理特征及预后.方法 回顾分析了广东省人民医院10年间诊断的55例EN-NK/TCL,复习其形态学特征、免疫表型及原位杂交检测结果,并进行临床资料的整理和随访.结果 患者男女比例为1.89:1,中位年龄38岁;初次就诊时病变部位以鼻腔及其邻近结构为主(85.5%).形态上瘤细胞以小到中等大细胞为主,血管中心浸润、血管破坏、凝固性坏死和凋亡小体常见.免疫表型CD20均为阴性;阳性率:CD3ε、CD4、CD5、CD8分别为100%(49/49)、7%(3/46)、8%(4/48)、63%(29/46);CD56为79%(42/53);T细胞内抗原-1和粒酶B均100%(49/49);bcl-2为17%(8/46);增殖指数:≤29%为30%(14/47),30%～59%为28%(13/47),≥60%为42%(20/47);bcl-2阳性病例增殖指数高;EBER 51例均阳性.41例获得临床资料,Ann Arbor分期Ⅰ/Ⅱ期为63.4%(26/41);87.8%(36/41)患者初诊时行为状态为1;31.8%(7/22)的患者血清乳酸脱氢酶(LDH)升高.获得27例随访资料,中位生存时间13个月,1年、2年及5年的生存率分别为52%、31%及20%.增殖指数≥60%的患者预后差.结论 EN-NK/TCL是一种成熟T细胞和NK细胞肿瘤,诊断时需要结合临床资料、形态学特征、免疫表型及EBER原位杂交结果.患者总体预后较差,增殖指数是独立的预后因素.

abstractsObjective To study the clinicopathologic features and prognostic factors of extranodal nasal type NK/T-cell lymphoma (EN-NK/TCL) in Chinese patients. Methods Fifty-five cases of EN-NK/ TCL diagnosed in Chinese patients during the period from 1998 to 2007 were studied by light microscopy, immunohistochemistry and in-situ hybridization. The follow-up information was analyzed. Results The male-to-female ratio was 1.89: 1. The median age of the patients was 38 years. The commonest sites of involvement included nasal cavity and adjoining tissue (85.5%). Histologically, EN-NK/TCL was composed of small to medium-sized lymphoid cells. Angiocentric and angiodestructive growth patterns, coagulative tumor necrosis and apoptotic bodies were frequently observed, Immunohistochemical study showed that CD20 ,the B-cell marker, was negative in all cases. The positiveity rates for T-cell markers CD3ε, CD4, CD5 and CD8 were 100% (49/49) ,7% (3/46) ,8% (4/48) and 63% (29/46) ,respectively. Most cases were also positive for NK-cell marker CD56 (79% 42/53). All cases expressed cytotoxie granule-associated proteins TIA-1 and granzyme B. Only 17% (8/46) of the cases were positive for anti-apoptotic protein bcl-2. The proliferation index, as demonstrated by Ki-67 immunostain, varied: 30% (14/47) with a low Ki-67 expression level(<or=29%), 28% (13/47) with a medium level (30%-59%) and 42% with a high level(>or=60%). There was a significant positive correlation between the bcl-2 positive expression and a high Ki-67 expression level. In-situ hybridization for EBV-encoded RNA was positive in all cases. Amongst the 41 cases with clinical information available, 63.4% presented with Ann Arbor stage Ⅰ to Ⅱ. The performance status score was 1 in 87.8% cases. High lactate dehydrogenase level was demonstrated in some patients(31.8%). Amongst the 27 cases with follow-up data available, the median survival was 13 months. The overall 1-year, 2-year and 5-year survival rates were 52%, 31% and 20%, respectively. In general, cases with high proliferation index carried poor prognosis. Conclusions EN-NK/TCL is a mature T-cell and NK-cell neoplasm which can be accurately diagnosed by histologic examination, immunohistochemical study and in-situ hybridization. The prognosis is usually not favorable. Proliferation index of the tumor represents an independent prognostic factor.