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结外鼻型NK/T细胞淋巴瘤(ENKTCL)是一种少见疾病,其分布具有明显的地域和种族特征,西方国家比较少见,多发生于亚洲、墨西哥及中南美洲,占所有非霍奇金淋巴瘤的5%～18%。按解剖部位被划分为上呼吸消化道NK/T细胞淋巴瘤(UAT-NKTCL)和非上呼吸消化道NK/T细胞淋巴瘤(NUAT-NKTCL)。在非上呼吸消化道病例中,皮肤往往是瘤体最常侵犯的器官。皮肤受累的结外鼻型NK/T细胞淋巴瘤称为皮肤NK/T细胞淋巴瘤(CNKTCL),包括了原发于皮肤以及原发于皮肤外的晚期ENKTCL皮肤浸润,但是不论是疾病原发或是继发的晚期皮肤浸润,其病程进展快,预后差,中位生存时间<12个月。侵袭性淋巴瘤常用的化学疗法方案如CHOP(环磷酰胺+多柔比星+长春新碱+泼尼松)或者CHOP类似方案以及对鼻部早期患者证实有效的局部放射治疗在皮肤NK/T患者中并未带来明显的生存获益,目前新的治疗方案还在探索中。随着肿瘤分子生物学的标靶干预和基因组研究的深入进展,靶向治疗有望为CNKTCL患者带来福音。本文将着重就CNKTCL治疗现状和预后加以综述。
Extranodal nasal type NK / T cell lymphoma (ENKTCL) is a rare disease with obvious geographical and ethnic characteristics. It is rare in western countries and occurs in Asia, Mexico and Central and South America, accounting for all non-Hodgkin’s Lymphoma of 5% to 18%. Anatomically divided into upper respiratory tract digestive tract NK / T-cell lymphoma (UAT-NKTCL) and non-upper gastrointestinal tract NK / T-cell lymphoma (NUAT-NKTCL). In cases of non-upper respiratory digestive tract, the skin is often the most frequently invaded organ of the tumor. Extranodal nasal type NK / T cell lymphomas involving the skin are called cutaneous NK / T cell lymphomas (CNKTCL) and include advanced ENKTCL skin infiltrates of the primary and extra-dermal but not primary Or secondary advanced skin infiltration, the course of its rapid progress, the prognosis is poor, the median survival time <12 months. Chemotherapy regimens commonly used in aggressive lymphomas such as CHOP (cyclophosphamide + doxorubicin + vincristine + prednisone) or CHOP-like regimens as well as local radiotherapy proven effective in early nasal patients on the skin NK / T Patients did not bring significant survival benefits, the current new treatment options are still under exploration. Targeted therapy is expected to bring gospel to patients with CNKTCL with the targeted intervention of tumor molecular biology and the progress of genome research. This article will focus on the status and prognosis of CNKTCL treatment to be reviewed.