2nd Department of Dermatology and Venereal Diseases, Attikon General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Expert Rev Clin Immunol. 2021 Jun;17(6):629-641. doi: 10.1080/1744666X.2021.1919085. Epub 2021 May 11.
: Cutaneous T-cell lymphoma (CTCL) is a rare non-Hodgkin's lymphoma, characterized by malignant T cells infiltrating the skin. CTCL exhibits vast heterogeneity which complicates diagnosis and therapeutic strategies. Current CTCL treatment includes skin-directed therapies (such as topical corticosteroid, topical mechlorethamine, topical bexarotene, ultraviolet phototherapy and localized radiotherapy), total skin electron beam therapy and systemic therapies. Elucidation of molecular and signaling pathways underlying CTCL pathogenesis leads to identification of innovative and personalized treatment schemes.: The authors reviewed the molecular and immunological aspects of CTCL with special focus on Mycosis Fungoides (MF), Sézary Syndrome (SS) and associated systemic treatment. A literature search was conducted in PubMed and Web of Science for peer-reviewed articles published until November 2020. Novel treatment approaches including retinoids, targeted therapies, immune checkpoint and JAK/STAT inhibitors, histones deacetylase (HDAC) and mTOR inhibitors as well as proteasome inhibitors, are discussed as potential therapeutic tools for the treatment of CTCL.: Novel therapeutic agents exhibit potential beneficial effects in CTCL patients of high need for therapy such as refractory early stage cutaneous and advanced stage disease. Therapeutic schemes employing a combination of novel agents with current treatment options may prove valuable for the future management of CTCL patients.
: 皮肤 T 细胞淋巴瘤(CTCL)是一种罕见的非霍奇金淋巴瘤,其特征是恶性 T 细胞浸润皮肤。CTCL 表现出巨大的异质性,这使得诊断和治疗策略变得复杂。目前 CTCL 的治疗包括皮肤靶向治疗(如局部皮质类固醇、局部氮芥、局部贝沙罗汀、紫外线光疗和局部放射治疗)、全身电子束治疗和全身治疗。阐明 CTCL 发病机制的分子和信号通路导致了创新和个性化治疗方案的确定。: 作者综述了 CTCL 的分子和免疫学方面,特别关注蕈样真菌病(MF)、塞扎里综合征(SS)和相关的全身治疗。在 PubMed 和 Web of Science 上对截至 2020 年 11 月发表的同行评议文章进行了文献检索。讨论了新型治疗方法,包括类视黄醇、靶向治疗、免疫检查点和 JAK/STAT 抑制剂、组蛋白去乙酰化酶(HDAC)和 mTOR 抑制剂以及蛋白酶体抑制剂,作为治疗 CTCL 的潜在治疗工具。: 新型治疗药物在高治疗需求的 CTCL 患者(如早期皮肤难治性和晚期疾病)中显示出潜在的有益效果。采用新型药物联合当前治疗方案的治疗方案可能对未来 CTCL 患者的管理具有重要价值。