Oncology (Williston Park). 2023 Feb 24;37(2):55-62. doi: 10.46883/2023.25920984.
Cutaneous T-cell lymphomas (CTCLs) are clinically heterogeneous T-cell lymphomas that arise in the skin and are characterized by their clinical and pathological features. This review will focus on mycosis fungoides (MF) and Sézary syndrome (SS), which represent 60% to 80% and less than 10% of CTCL cases, respectively. While most patients with MF present with patches and plaques and can be successfully treated with skin-directed therapies, a minority of patients progress from early to advanced stages or undergo large cell transformation. SS is defined as erythroderma, lymphadenopathy, and more than 1000 circulating atypical T-cells/uL with cerebriform nuclei. It has a poor overall survival of 2.5 years. Given the overall rarity of CTCLs, it is notable that clinical trials of treatments for MF/SS have been successfully completed, resulting in FDA approvals of novel therapies with increasing overall response rates. This review outlines the current multidisciplinary approach to diagnosing and treating MF/SS, with a focus on combining skin-directed therapies with emerging targeted and investigational systemic therapies. Integrating these anticancer therapies with skin care and bacterial decolonization is critical for comprehensive management. Curing patients with MF/SS may be possible by using a personalized medicine approach including novel combination strategies, restoration of T helper 1 cytokines, and avoidance of immunosuppressive regimens.
皮肤 T 细胞淋巴瘤（CTCLs）是临床上异质性的 T 细胞淋巴瘤，起源于皮肤，其特征为临床和病理特征。本综述将重点介绍蕈样真菌病（MF）和赛泽里综合征（SS），它们分别占 CTCL 病例的 60%至 80%和小于 10%。虽然大多数 MF 患者表现为斑块和斑块，并且可以通过皮肤靶向治疗成功治疗，但少数患者从早期进展为晚期或发生大细胞转化。SS 定义为红皮病、淋巴结病和循环中超过 1000 个具有脑回核的异常 T 细胞/uL。它的总生存率为 2.5 年。鉴于 CTCLs 的总体罕见性，值得注意的是，MF/SS 治疗的临床试验已经成功完成，导致 FDA 批准了新型治疗方法，总体反应率不断提高。本综述概述了目前用于诊断和治疗 MF/SS 的多学科方法，重点是将皮肤靶向治疗与新兴的靶向和研究性全身治疗相结合。将这些抗癌疗法与皮肤护理和细菌去定植相结合对于综合管理至关重要。通过使用包括新型联合策略、辅助性 T 细胞 1 细胞因子恢复和避免免疫抑制方案在内的个性化医学方法，可能治愈 MF/SS 患者。
