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多灶性原发性皮肤结外NK/T细胞淋巴瘤,鼻型

Multifocal primary cutaneous extranodal NK/T lymphoma nasal type.

作者信息

Vasconcelos Pedro de, Ferreira Cristina, Soares-Almeida Luís, Filipe Paulo

机构信息

Hospital de Santa Maria, Lisboa, Portugal.

出版信息

An Bras Dermatol. 2016 Apr;91(2):219-21. doi: 10.1590/abd1806-4841.20164090.

Abstract

Nasal type extranodal NK/T-cell lymphoma is a distinct entity according to the World Health Organization classification. Although 60% to 90% of patients with this disease present with a destructive mass in the midline facial tissues, it may also primarily or secondarily involve extranasal sites, like the skin. We report the case of a 77-year-old patient that came to our department with erythematous plaques of the right leg and eczematous lesions of the trunk. These lesions were biopsied and the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He was treated with multi-agent systemic chemotherapy but died 5 months after diagnosis. This case highlights the rarity and variability of cutaneous features of this disease and its aggressive course and poor prognosis.

摘要

根据世界卫生组织的分类,鼻型结外NK/T细胞淋巴瘤是一种独特的疾病实体。尽管该疾病60%至90%的患者表现为中线面部组织的破坏性肿块,但它也可能原发或继发累及鼻外部位,如皮肤。我们报告了一例77岁的患者,该患者因右腿红斑性斑块和躯干湿疹样病变前来我院就诊。对这些病变进行了活检,患者被诊断为鼻型结外NK/T细胞淋巴瘤。他接受了多药全身化疗,但在诊断后5个月死亡。该病例突出了这种疾病皮肤特征的罕见性和变异性,以及其侵袭性病程和不良预后。

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