Primary cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. CTCL subtypes demonstrate a variety of clinical, histological, and molecular features, and can follow an indolent or a very aggressive course. The underlying pathogenetic mechanisms are not yet entirely understood. The pathophysiology of CTCL is complex and a single initiating factor has not yet been identified. Diagnosis is based on clinicopathological correlation and requires an interdisciplinary team. Treatment decision is made based on short-term and long-term goals. Therapy options comprise skin-directed therapies, such as topical steroids or phototherapy, and systemic therapies, such as monoclonal antibodies or chemotherapy. So far, the only curative treatment approach is allogeneic haematopoietic stem cell transplantation. Novel therapies, such as chimeric antigen receptor T cells, monoclonal antibodies or small molecules, are being investigated in clinical trials. Patients with CTCL have reduced quality of life and a lack of effective treatment options. Further research is needed to better identify the underlying mechanisms of CTCL development and course as well as to better tailor treatment strategies to individual patients.

原发性皮肤 T 细胞淋巴瘤 (CTCL) 是一组异质性淋巴瘤，存在于皮肤中，在诊断时没有皮外疾病的证据。CTCL 亚型表现出多种临床、组织学和分子特征，并且可以遵循惰性或非常激进的过程。潜在的发病机制尚不完全清楚。CTCL 的病理生理学很复杂，单一的起始因素尚未确定。诊断基于临床病理学相关性，需要跨学科团队。治疗决定是根据短期和长期目标做出的。治疗选择包括皮​​肤导向治疗，如局部类固醇或光疗，以及全身治疗，如单克隆抗体或化疗。至今，唯一的治疗方法是异基因造血干细胞移植。新疗法，如嵌合抗原受体 T 细胞、单克隆抗体或小分子，正在临床试验中进行研究。CTCL 患者的生活质量下降，缺乏有效的治疗选择。需要进一步研究以更好地确定 CTCL 发展和病程的潜在机制，以及更好地为个体患者制定治疗策略。