Roccuzzo Gabriele, Giordano Silvia, Fava Paolo, Pileri Alessandro, Guglielmo Alba, Tonella Luca, Sanlorenzo Martina, Ribero Simone, Fierro Maria Teresa, Quaglino Pietro
Department of Medical Sciences, Section of Dermatology, University of Turin, Turin, Italy.
Dermatology-IRCCS Policlinico di Sant'Orsola Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
Front Oncol. 2021 Aug 16;11:733770. doi: 10.3389/fonc.2021.733770. eCollection 2021.
Primary cutaneous T-cell lymphomas (PCTCL) are the most common types of cutaneous lymphomas, with Mycosis fungoides as the most frequent subtype. Besides early stages which usually have a good prognosis, advanced stages remain a great therapeutic challenge with low survival rates. To date, none of the currently available therapeutic options have significantly improved the outcomes of advanced cutaneous lymphomas. Recent studies have demonstrated that immune-checkpoint molecules, such as PD-1 and CTLA-4, play part in the proliferation pathways of neoplastic T-cells, as well as in other tumors. Hence, the potential role of immune-checkpoint-inhibitors in treating cutaneous lymphomas has been investigated in the last years. Herein, we outline the current knowledge regarding the role of immune-checkpoint molecules in PCTCL, their signaling pathways, microenvironment and therapeutic inhibition rationale. Moreover, we review the published data on immunotherapies in PCTCL and summarize the currently ongoing clinical trials in this field.
原发性皮肤T细胞淋巴瘤（PCTCL）是最常见的皮肤淋巴瘤类型，蕈样肉芽肿是最常见的亚型。除了通常预后良好的早期阶段外，晚期阶段仍然是一个巨大的治疗挑战，生存率较低。迄今为止，目前可用的治疗选择均未显著改善晚期皮肤淋巴瘤的治疗结果。最近的研究表明，免疫检查点分子，如PD-1和CTLA-4，在肿瘤性T细胞的增殖途径以及其他肿瘤中发挥作用。因此，近年来已经研究了免疫检查点抑制剂在治疗皮肤淋巴瘤中的潜在作用。在此，我们概述了关于免疫检查点分子在PCTCL中的作用、其信号通路、微环境和治疗抑制原理的当前知识。此外，我们回顾了PCTCL免疫治疗的已发表数据，并总结了该领域目前正在进行的临床试验。
