Rozas-Muñoz Eduardo, Gallardo Fernando, Pujol Ramon Maria, Pérez-Ferriols Amparo, Servitje Octavio, Estrach Teresa, Bastida Jesus, Román Concha, Palacio-Aller Lucia, Gil Inmaculada, Martí Rosa Maria, Vidal-Sarró David, García-Muret María Pilar
Dermatology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Dermatology Department, Parc de Salut Mar-Hospital del Mar, Barcelona, Spain.
Eur J Dermatol. 2018 Feb 1;28(1):64-70. doi: 10.1684/ejd.2017.3205.
Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is an aggressive lymphoma with a very low incidence in western populations.
To review the clinicopathological features and outcome of a multicentre series of ENKTL in Spain.
MATERIALS & METHODS: A multicentre retrospective study was performed based on cases of ENKTL, collected from 1995 to 2004, from 12 dermatology departments included in the Spanish Lymphoma Study Group. The clinical, histopathological, and evolutive features of all these cases were reviewed.
Eighteen patients (three male, 15 female) with median age of 67 years were included in the study. The onset of lesions occurred in the nasal region in 11 patients and on the skin outside this region in the remaining cases. The observed lesions were clinically heterogeneous, corresponding to papules, plaques, and nodules, with or without ulceration. All patients except four received different polychemotherapy regimens, either alone (n = 11) or in combination with radiotherapy (n = 4). After a variable follow-up period (1-36 months), only two patients remained alive. One patient was recently diagnosed (four months ago) with ENKTL in the nasal region and the other presented with skin-limited disease. The median overall survival was 9.5 months.
The results of this retrospective survey confirm that ENKTL is a rare subtype of lymphoma in the Spanish population. All patients showed an aggressive clinical course and poor prognosis, regardless of the initial clinical presentation. Prospective data on larger series of patients treated homogenously are needed to establish the best treatment modality.
结外鼻型自然杀伤/T细胞淋巴瘤（ENKTL）是一种侵袭性淋巴瘤，在西方人群中发病率极低。
回顾西班牙多中心系列ENKTL的临床病理特征及预后。
基于1995年至2004年西班牙淋巴瘤研究组纳入的12个皮肤科的ENKTL病例进行多中心回顾性研究。对所有这些病例的临床、组织病理学及演变特征进行了回顾。
18例患者（3例男性，15例女性）纳入研究，中位年龄67岁。11例患者病变首发于鼻区，其余患者病变位于该区域以外的皮肤。观察到的病变在临床上具有异质性，表现为丘疹、斑块和结节，有或无溃疡。除4例患者外，所有患者均接受了不同的多药化疗方案，单独化疗（n = 11）或联合放疗（n = 4）。经过不同的随访期（1 - 36个月），仅2例患者存活。1例患者最近（4个月前）被诊断为鼻区ENKTL，另1例患者表现为皮肤局限性病变。中位总生存期为9.5个月。
这项回顾性调查结果证实，ENKTL在西班牙人群中是一种罕见的淋巴瘤亚型。所有患者均表现出侵袭性临床病程及不良预后，无论初始临床表现如何。需要关于更多接受统一治疗的患者系列的前瞻性数据来确定最佳治疗方式。
