Department of Clinical Hematology, Université Sorbonne Paris Cité, Hôpital Necker, Paris, France.
Division of Hematology/Oncology, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL, USA.
Adv Ther. 2018 Feb;35(2):135-152. doi: 10.1007/s12325-018-0658-4. Epub 2018 Feb 6.
Adult T-cell leukemia-lymphoma (ATL), a rare and aggressive T-cell malignancy caused by human T-cell lymphotropic virus type 1 (HTLV-1), is associated with a poor prognosis. Evidence-based standard treatment options are lacking and outcomes are generally unsatisfactory, particularly for patients with relapsed or refractory disease. Continued research is contributing to changing treatment landscape as a number of existing and investigational agents are evaluated. We describe the epidemiology of HTLV-1 and ATL, discuss the biology behind the disease, review current treatment practices and guidelines, and provide an overview of emerging therapies in ATL, with a focus on those for relapsed or refractory disease.
成人 T 细胞白血病-淋巴瘤（ATL）是一种由人类 T 细胞白血病病毒 1 型（HTLV-1）引起的罕见且侵袭性的 T 细胞恶性肿瘤，预后较差。目前缺乏基于循证的标准治疗选择，治疗效果通常不理想，尤其是对于复发或难治性疾病的患者。随着许多现有和正在研究的药物进行评估，持续的研究正在改变治疗格局。我们描述了 HTLV-1 和 ATL 的流行病学，讨论了该疾病背后的生物学，回顾了目前的治疗实践和指南，并概述了 ATL 中的新兴治疗方法，重点是针对复发或难治性疾病的治疗方法。
