研究动态
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皮肤T细胞淋巴瘤:现有和新兴疗法。

Cutaneous T-Cell Lymphoma: Current and Emerging Therapies.

发表日期:2023 Feb 24
作者: Julia Dai, Madeleine Duvic
来源: Cellular & Molecular Immunology

摘要:

皮肤T细胞淋巴瘤(CTCLs)是一种临床异质性T细胞淋巴瘤,在皮肤中发生,并以其临床和病理特征为特征。本综述将重点讨论霉菌病样肿块(MF)和塞扎里综合征(SS),它们分别代表CTCL病例的60%至80%和不到10%。虽然大多数MF患者出现斑块和板块,可以通过皮肤定向治疗成功治疗,但少数患者从早期进展到晚期或经历大细胞转化。SS被定义为红皮病,淋巴结肿大和超过1000个带有脑纹核的异常T细胞/uL。它的总体生存期为2.5年。鉴于CTCL的整体罕见性,值得注意的是,针对MF / SS的治疗临床试验已经成功完成,导致FDA批准了新颖治疗方法,总体反应率逐渐增加。本综述概述了目前多学科诊断和治疗MF / SS的方法,重点是将皮肤定向治疗与新兴的靶向和调查性全身治疗结合起来。将这些抗癌疗法与皮肤护理和细菌去除相结合对于综合管理至关重要。通过使用个性化医学方法包括新的联合策略,修复T辅助1细胞因子,并避免免疫抑制剂方案,可能可以治愈MF / SS患者。
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.