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,改善患者的生存 Objective To retrospectively analyze the clinical features, treatment and prognosis of peripheral T-cell lymphoma occurring in the nasal and nasopharyngeal parts and to explore the improvement of the treatment methods. Methods One hundred and twelve patients were diagnosed by pathology and immunohistochemistry [of which 39 were clearly CD56 (+) NK / T cell lymphomas]. At the time of diagnosis, the median age was 46 years. There were 84 males and 28 females. The median duration of 4 months. Local lesions involving the nasal cavity (88 cases) and the nasopharynx (50 cases), as well as the adjacent parts, 83 cases of ultra-cavity. Clinical stage Ⅰ_E / Ⅱ_E lesions accounted for 91.1%. International prognostic index (IPI) less than 2 points accounted for 78.8%. Accept, put comprehensive treatment of 72 cases, chemotherapy alone in 32 cases, 3 cases of radiotherapy alone, 5 cases were untreated. Results The median survival time of patients was 42 months. The first course of treatment complete remission (CR) rate of 65.1%, pre-chemotherapy CR rate of 34.4%. The local tumor control rate was 50.5% and the median tumor progression time (TIP) was 11 months. More than 30% of the patients had evidence of clear systemic spread. The TTP of the superficial lesions was shorter (r _ (?) = - 0.191, P = 0.024). The 3-year progression-free survival rate and overall survival rate were 38.8% and 52.4% respectively. The 5-year progression-free survival rate and overall survival rate were 34.9% and 44.8% respectively. Univariate analysis showed that the survival of favorable factors, including duration of not less than 3 months, early lesions, non-NK / T cell pathology, no skin infiltration, IPI low risk, first-course chemotherapy CR, radiation therapy, Local control. Multivariate analysis showed that the course of disease was not less than 3 months (P = 0.011), non-NK / T cell pathological types (P = 0.007) (P = 0.000) was a favorable survival prognostic factor. Conclusions Nasal and nasopharyngeal peripheral T-cell lymphoma are mostly early lesions when diagnosed. However, some patients may present with highly aggressive and poor prognosis, including CD56 (+) NK / T cell lymphoma. Radiotherapy, comprehensive treatment as the main treatment has yet to be improved in order to improve the treatment response rate and improve patient survival