Pelcovits Ari, Ollila Thomas A, Olszewski Adam J
Department of Medicine, Alpert Medical School of Brown University, Providence, RI, USA.
Division of Hematology-Oncology, Rhode Island Hospital, Providence, RI, USA.
Cancer Manag Res. 2023 Sep 6;15:989-998. doi: 10.2147/CMAR.S330908. eCollection 2023.
Cutaneous T-Cell Lymphoma (CTCL) is a heterogenous disease that consists of distinct clinicopathologic entities and presentations requiring a unique and expert approach to management. The most common subtype is mycosis fungoides, in which local disease has an excellent prognosis and is often managed with topical therapy alone. More extensive cutaneous involvement as well as involvement of lymph nodes and the peripheral blood (Sezary syndrome) require systemic therapies. Recent years have brought an expansion of therapeutic options, specifically with immune-based approaches that were developed using the knowledge gained regarding the biology and molecular pathology of CTCL. Previous systemic therapies such as retinoids, histone deacetylase inhibitors, and chemotherapeutic agents come with significant toxicity and only short-term response. Newer agents such as mogamulizumab and brentuximab vedotin use a targeted immune-based approach leading to longer periods of response with less systemic toxicity. While still in its infancy, the use of immune checkpoint inhibitors such as nivolumab and pembrolizumab appears promising, and while their current clinical application is limited, early data suggest possible future areas for research of immune manipulation to treat CTCL. Herein, we review these novel immune-based treatment strategies, their superiority over prior systemic options, and the ongoing need for further research and clinical trial enrollment.
皮肤T细胞淋巴瘤（CTCL）是一种异质性疾病，由不同的临床病理实体和表现组成，需要独特的专业管理方法。最常见的亚型是蕈样肉芽肿，其中局部疾病预后良好，通常仅采用局部治疗。更广泛的皮肤受累以及淋巴结和外周血受累（塞扎里综合征）则需要全身治疗。近年来，治疗选择有所扩展，特别是基于免疫的方法，这些方法是利用从CTCL生物学和分子病理学中获得的知识开发的。以前的全身治疗方法，如维甲酸、组蛋白去乙酰化酶抑制剂和化疗药物，具有显著的毒性，且仅能产生短期反应。新型药物，如莫加莫拉单抗和本妥昔单抗，采用基于免疫的靶向方法，可产生更长时间的反应，且全身毒性较小。虽然免疫检查点抑制剂，如纳武单抗和派姆单抗的应用仍处于起步阶段，但似乎前景广阔，尽管它们目前的临床应用有限，但早期数据表明，免疫调控治疗CTCL可能是未来的研究领域。在此，我们综述这些基于免疫的新型治疗策略、它们相对于先前全身治疗方案的优势，以及持续开展进一步研究和临床试验入组的必要性。
