112例鼻和鼻咽外周T细胞淋巴瘤患者的疗效和预后分析

Treatment outcome and prognosis of 112 patients with nasal and nasopharyngeal peripheral T cell lymphomas

目的对发生于鼻腔和鼻咽部位的外周 T 细胞淋巴瘤的临床特点、治疗和预后情况进行回顾分析,并探讨治疗方法的改进。方法 112例患者均经病理和免疫组化证实诊断[其中39例明确为 CD56(+)的 NK/T 细胞淋巴瘤],确诊时中位年龄46岁,男84例,女28例,疗前病程中位数4个月。局部病变累及部位包括鼻腔(88例)和鼻咽(50例)以及临近部位,超腔者83例。临床分期Ⅰ_E/Ⅱ_E 期病变占91.1%。国际预后指数(IPI)小于2分者占78.8%。接受化、放综合治疗72例,单纯化疗32例,单纯放射治疗3例,5例未治。结果存活患者中位随访时间42个月。首程治疗后完全缓解(CR)率65.1%,先期化疗的 CR 率为34.4%。局部肿瘤控制率为50.5%,中位肿瘤进展时间(TIP)为11个月,超过30%的患者有明确全身播散的证据。超腔病变的 TTP 较短(r_(?)=-0.191,P=0.024)。全组3年无进展生存率和总生存率分别为38.8%和52.4%,5年无进展生存率和总生存率分别为34.9%和44.8%。单因素分析显示生存有利因素包括病程不少于3个月、早期病变、非 NK/T 细胞病理类型、无皮肤浸润、IPI 低危、首程化疗达 CR、放射治疗、首程治疗达 CR 和局部控制。多因素分析显示在病情和治疗因素中,病程不少于3个月(P=0.011)、非 NK/T 细胞病理类型(P=0.007)、首程化疗达 CR(P=0.008)和放射治疗(P=0.000)是生存有利的预后因素。结论鼻和鼻咽外周 T 细胞淋巴瘤虽然确诊时多为早期病变,但部分患者可表现为高度侵袭性、预后不良,其中包括 CD56(+)的NK/T 细胞淋巴瘤。放、化综合治疗作为主要的治疗手段尚待改进,以提高治疗缓解率。

Objective To retrospectively analyze the treatment outcomes and prognostic factors of nasal and nasopharyngeal peripheral T cell lymphomas（PTCL） patients. Methods One hundred and twelve patients with pathologically confirmed nasal and nasopharyngeal PTCL were included, among which 39 were CD56（ ＋ ） NK/T cell lymphomas. The median pre-treatment disease course was 4 months. 84 were males and 28 females median age was 46 years. The tumors mainly involved nasal cavity（ 88 cases） and/or naso- pharynx（50 eases） and adjacent structures, and 83 cases with extra-cavity diseases. 91.1% of the patients had Ann Arbor ⅠE/ⅡE diseases. The International Prognostic Indices （IPI） were less than 2 scores in 78.8% of the patients. Seventy two patients received combined chemo-radiotherapy, 32 chemotherapy only, 3 radiotherapy only and 5 no any treatment. Results Median follow-up duration was 42 months. Chemotherapy achieved a complete remission（CR） rate of 34.4% for initial treatment, and of 65. 1% after primary treatment. The local tumor controlled rate was 50.5% , and the median time to tumor progression（TTP） was 11 months. There were evidences of systemic relapse in more than 30% of the patients. The extra-cavity tumors usually had a shorter TTP （r = -0. 191 ,P =0. 024）. The progress-free survival and overall survival rates were 38.8% and 52.4% at 3 years, and 34.9% and 44.8% at 5 years respectively. Univariate analysis ,showed that favorable prognostic factors for survival were pre-treatment course 〉 3 months, earlier clinical stage, non NK/T lymphoma, no skin involvement, lower IPI, CR after initial chemotherapy, radiotherapy, CR ＇after primary treatment and local tumor controlled. Multivariate analysis showed that, pre-treatment course 〉 3 months （ P = 0.011 ）, non NK/T lymphoma （ P = 0. 007 ）, CR after initial ehemotherapy（ P = 0. 008 ）and radiotherapy （ P = 0. 000） were favorable prognostic factors for survival. Conclusions Although most nasal and nasopharyngeal peripheral T- cell lymphomas were diagnosed at early stage diseases, some of them were highly aggressive with poor prognose, particularly CD56 （ ＋ ） NK/T cell lymphomas. Combination chemo/radiotherapy, though remained principal treatments, more effective therapeutic modalities are expected.