Department of Dermatology, University Hospital Zurich, CH-8091 Zurich, Switzerland.
Acta Derm Venereol. 2018 Oct 10;98(9):835-841. doi: 10.2340/00015555-2950.
Rare T- or NK-cell lymphomas with cutaneous manifestation may display a highly aggressive clinical course and major diagnostic/therapeutic challenges. This report describes our experiences with different lymphomas of this rare category and the therapeutic options used. This retrospective, descriptive, monocentric, cross-sectional case study, identified 4 rare aggressive T-/NK-cell lymphomas with manifestation in the skin, which were diagnosed in a tertiary care centre over a period of 4 years. Two patients had an Epstein-Barr virus-associated extranodal NK/T-cell lymphoma and 2 patients had a primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma. Concomitant extracutaneous involvement was observed in 2 of all 4 patients. Two patients had fulminant disease progression and resistance to chemotherapy. Two patients underwent allogeneic haematopoietic stem cell transplantation, which resulted in one complete remission and one partial remission. This report emphasizes the importance of an early diagnostic work-up and a prompt aggressive therapeutic approach.
罕见的具有皮肤表现的 T 细胞或 NK 细胞淋巴瘤可能具有高度侵袭性的临床病程和重大的诊断/治疗挑战。本报告描述了我们在这一罕见类别中不同淋巴瘤的经验以及所使用的治疗选择。这项回顾性、描述性、单中心、横断面病例研究确定了 4 例罕见的具有侵袭性的 T/NK 细胞淋巴瘤，这些淋巴瘤在 4 年内于一家三级护理中心以皮肤表现的形式被诊断出来。2 例患者患有 EBV 相关结外 NK/T 细胞淋巴瘤，2 例患者患有原发性皮肤 CD8+侵袭性表皮亲噬细胞毒性 T 细胞淋巴瘤。所有 4 例患者中均有 2 例同时存在皮肤外受累。2 例患者疾病迅速进展且对化疗有耐药性。2 例患者接受了异基因造血干细胞移植，其中 1 例患者完全缓解，1 例患者部分缓解。本报告强调了早期诊断和积极治疗方法的重要性。
