原发上呼吸消化道外结外NK/T细胞淋巴瘤的临床特征及生存分析

doi:10.3760/cma.j.issn.1673-422X.2020.03.006

摘要/Abstract

摘要：

目的分析原发上呼吸消化道外结外NK/T细胞淋巴瘤(EUADT-ENKTCL)患者的临床特征及生存预后情况。方法收集自2000年至2015年SEER数据库登记的779例结外NK/T细胞淋巴瘤(ENKTCL)患者的临床及生存资料,其中原发上呼吸消化道结外NK/T细胞淋巴瘤(UADT-ENKTCL)患者622例,EUADT-ENKTCL患者157例。对EUADT-ENKTCL患者的临床特征、生存情况及预后因素进行分析。结果 EUADT-ENKTCL患者中位发病年龄为55岁,男女比例为1.85∶1。相较于UADT-ENKTCL,EUADT-ENKTCL患者中T细胞来源(χ ²=18.916,P<0.001)、晚期病变(χ ²=63.669,P<0.001)、伴随B症状(χ ²=14.657,P=0.001)以及接受单纯化疗(χ ²=71.412,P<0.001)更多见。EUADT-ENKTCL可原发于全身不同器官组织,其中皮肤或皮下软组织最多见(38.2%,60/157),其次依次为胃肠道(18.5%,29/157)、肺/胸膜(13.4%,21/157)、睾丸(6.4%,10/157)和眼眶(5.1%,8/157)等。EUADT-ENKTCL患者中位总生存时间(OS)和5年OS率分别为8.0个月和24.0%,明显劣于UADT-ENKTCL患者(31.0个月和43.5%; χ ²=32.080,P<0.001)。单因素分析结果提示晚期、肺原发病变的EUADT-ENKTCL患者预后更差,多因素分析证实临床分期是影响OS的预后因素(Ⅲ~Ⅳ期 vs.Ⅰ~Ⅱ期,HR=2.078,95%CI为1.335~3.234,P=0.001)。结论 EUADT-ENKTCL是一类高度侵袭性疾病,与UADT-NKTCL相比,存在临床特征的差异性且长期生存不理想。

关键词: 淋巴瘤, 结外NK-T细胞, 生存, 预后, 上消化呼吸道外

Abstract:

Objective To analyze the clinical characteristics, survival outcome and prognostic factors of patients with extra-upper aerodigestive tract extranodal NK/T-cell lymphoma (EUADT-ENKTCL). Methods We collected the clinical data of 779 patients with ENKTCL who were registered in SEER (Surveillance, Epidemiology, and End Results) database from 2000 to 2015 including 622 cases diagnosed with upper-aerodigestive tract extranodal NK/T-cell lymphoma (UADT-ENKTCL) and 157 cases with EUADT-ENKTCL. Clinical characteristics, survival outcome and prognostic factors were analyzed in EUADT-ENKTCL patients. Results The median age of patients with EUADT-ENKTCL was 55. The ratio of male to female was 1.85∶1. Compared with UADT-ENKTCL, patients with EUADT-ENKTCL accounted a higher proportion of malignant T-cell origin (χ ²=18.916, P<0.001), advanced stage disease (χ ²=63.669, P<0.001), accompanied B symptoms (χ ²=14.657, P=0.001) and receiving chemotherapy alone (χ ²=71.412, P<0.001). EUADT-ENKTCL could be originated in different organs and tissues throughout the body, among which the skin or subcutaneous soft tissues was the most common locations (38.2%, 60/157), followed by gastrointestinal tract (18.5%, 29/157), lung/pleura (13.4%, 21/157), testis (6.4%, 10/157), orbit (5.1%, 8/157) and others. The median overall survival (OS) was 8.0 months and 5-year OS rate was 24.0% for the EUADT-ENKTCL cohort, which were much inferior to those of UADT-ENKTCL patients (31.0 months and 43.5%; χ ²=32.080, P<0.001). Univariate analysis showed that patients with advanced stage or primary lung disease of EUADT-ENKTCL had unfavorable OS. Stage was a prognostic factor of OS via multivariate analysis (Ⅲ/Ⅳ vs. Ⅰ/Ⅱ, HR=2.078, 95%CI: 1.335-3.234, P=0.001). Conclusion EUADT-ENKTCL is a highly aggressive malignancy. Compared with UADT-ENKTCL, EUADT-ENKTCL patients have different clinical characteristics and significantly inferior OS.

Key words: Lymphoma, extranodal NK-T-cell, Survival, Prognosis, Extra-upper aerodigestive tract

齐菲, 董梅. 原发上呼吸消化道外结外NK/T细胞淋巴瘤的临床特征及生存分析[J]. 国际肿瘤学杂志, 2020, 47(3): 157-163.

Qi Fei, Dong Mei. Clinical characteristics and survival analysis of extra-upper aerodigestive tract extranodal NK/T-cell lymphoma[J]. Journal of International Oncology, 2020, 47(3): 157-163.

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临床特征	UADT-ENKTCL 组(n=622)	EUADT-ENKTCL 组(n=157)
年龄(岁)
≤60	398(64.0)	90(57.3)	2.378	0.123
>60	224(36.0)	67(42.8)
性别
女	231(37.1)	55(35.0)	0.239	0.624
男	391(62.9)	102(65.0)
种族
白种人	436(70.1)	112(71.3)
黑种人	23(3.7)	6(3.8)	0.123	0.940
其他	163(26.2)	39(24.8)
细胞起源
NK细胞	459(73.8)	88(56.1)
T细胞	133(21.4)	57(36.3)	18.916	<0.001
其他	30(4.8)	12(7.6)
分期
Ⅰ~Ⅱ期	473(76.0)	72(45.9)
Ⅲ~Ⅳ期	126(20.3)	60(38.2)	63.669	<0.001
不详	23(3.7)	25(15.9)
B症状
无	304(48.9)	51(32.5)
有	240(38.6)	75(49.0)	14.657	0.001
不详	78(12.5)	31(17.8)
治疗模式
无	91(14.6)	40(25.5)
单纯化疗	143(23.0)	77(49.0)	71.412	<0.001
单纯放疗	107(17.2)	16(10.2)
放化疗联合	281(45.2)	24(15.3)

特征	单因素分析				多因素分析
特征	HR值	95%CI	P值		HR值	95%CI	P值
年龄(>60岁vs.≤60岁)	1.432	1.557~1.772	0.001	1.311		1.041~1.650	0.021
性别(男vs.女)	1.070	0.860~1.331	0.543	-		-	-
种族
白种人	1			1
黑种人	2.513	0.994~2.303	0.054	1.346		0.853~2.124	0.202
其他	0.981	0.776~1.267	0.944	0.923		0.721~1.182	0.527
细胞来源(T vs. NK细胞)	1.173	0.985~1.397	0.073	1.096		0.840~1.429	0.500
分期(Ⅲ~Ⅳ vs.Ⅰ~Ⅱ)	2.133	1.693~2.687	<0.001	1.839		1.048~3.228	0.034
B症状(有vs.无)	1.242	1.128~1.368	<0.001	1.115		0.872~1.426	0.386
原发部位(鼻腔外vs.鼻腔)	1.531	1.237~1.895	<0.001	1.427		1.122~1.815	0.004
治疗模式
无	1			1
单纯化疗	1.124	0.817~1.547	0.473	1.076		0.768~1.509	1.076
单纯放疗	0.705	0.493~0.956	0.047	0.838		0.572~1.227	0.363
放化疗联合	0.433	0.316~0.593	<0.001	0.514		0.347~0.719	<0.001

特征	HR值	95%CI	P值
年龄(>60岁vs.≤60岁)	1.274	0.900~1.803	0.172
性别(男vs.女)	0.828	0.578~1.187	0.305
种族
白色	1
黑色	0.987	0.362~2.691	0.980
其他	0.914	0.595~1.404	0.682
细胞来源(T vs. NK细胞)	1.012	0.701~1.460	0.957
分期(Ⅲ~Ⅳ vs.Ⅰ~Ⅱ)	1.855	1.259~2.735	0.001
B症状(有 vs.无)	1.274	0.834~1.947	0.263
原发部位
皮肤	1
肺	1.692	1.014~2.823	0.044
胃肠道	0.976	0.588~1.620	0.924
睾丸	1.049	0.496~2.218	0.900
眼眶	0.935	0.401~2.181	0.876
治疗模式
无	1
单纯化疗	0.848	0.555~1.298	0.449
单纯放疗	0.824	0.440~1.534	0.545
放化疗联合	0.578	0.325~1.027	0.062