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：CD3e、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）；bel-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。
Objective 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. Angiocentrlc and angiodestructive growth patterns, coagulative tumor necrosis and apoptotic bodies were frequently observed. Immunohistoehemical study showed that CD20,the B-cell marker, was negative in all cases. The positiveity rates for T-cell markers CD3e, 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 cytotoxic granule-associated proteins TIA-1 and granzyme B. Only 17% （8/46） of the cases were positive for anti-apoptotie 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
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2009年第4期237-242,共6页
Chinese Journal of Pathology
基金
广东省自然科学基金（06020908）
广东省人民医院科研项目（Y200521）
关键词
淋巴瘤
T细胞
外周
诊断
预后
Lymphoma, T-cell, peripheral
Diagnosis
Prognosis
