成人T细胞白血病-淋巴瘤被定义为由人类T细胞白血病病毒I型引起的外周T细胞淋巴瘤。成人T细胞白血病-淋巴瘤分为慢性（有利的慢性或潜行性）和侵袭性（急性、淋巴瘤或不利的慢性）两种类型。本综述讨论了成人T细胞白血病-淋巴瘤患者的治疗进展和治疗成人T细胞白血病-淋巴瘤的未满足问题。对于慢性成人T细胞白血病-淋巴瘤，建议采用观察等待策略，直到疾病发展为侵袭性成人T细胞白血病-淋巴瘤。对于侵袭性成人T细胞白血病-淋巴瘤，建议采用多药物化疗，可选择同种异基因造血干细胞移植。然而，许多成人T细胞白血病-淋巴瘤患者会复发，预后较差。最近，新药，包括莫伐单抗、雷那利胺、布伦妥他蛋白-维多汀、吐西冬尾苷和瓦勒霉素，已经获得批准，用于治疗复发性或难治性侵袭性成人T细胞白血病-淋巴瘤的患者，且莫伐单抗与多药物化疗或布伦妥他蛋白-维多汀与环磷酰胺、多柔比星和泼尼松联合应用已在日本获得批准，用于治疗未经治疗的侵袭性成人T细胞白血病-淋巴瘤患者。值得注意的是，最近有报道称成人T细胞白血病-淋巴瘤患者的年龄趋向老龄化，目前尚未为年龄较大的成人T细胞白血病-淋巴瘤患者确立标准治疗。必须从前瞻性临床试验中获取新的证据，以改善成人T细胞白血病-淋巴瘤患者的预后。（翻译© 国际牛津大学出版社，2023。保留所有权利。如需权限，请发送电子邮件至：journals.permissions@oup.com。）

Adult T-cell leukemia-lymphoma is defined as peripheral T-cell lymphoma caused by the human T-cell leukemia virus type I. Adult T-cell leukemia-lymphoma is classified into indolent (favorable chronic or smoldering) or aggressive (acute, lymphoma or unfavorable chronic) types. This review discusses the therapeutic developments for patients with adult T-cell leukemia-lymphoma and unmet issues in treating adult T-cell leukemia-lymphoma. For indolent adult T-cell leukemia-lymphoma, a watchful waiting strategy is recommended until the disease progresses to aggressive adult T-cell leukemia-lymphoma. For aggressive adult T-cell leukemia-lymphoma, multi-agent chemotherapy with or without allogeneic hematopoietic stem cell transplantation has been recommended. However, many patients with adult T-cell leukemia-lymphoma relapse, and their prognosis is poor. Recently, novel agents, including mogamulizumab, lenalidomide, brentuximab vedotin, tucidinostat and valemetostat, have been approved for patients with relapsed or refractory aggressive adult T-cell leukemia-lymphoma, and the combination of mogamulizumab with multi-agent chemotherapy or brentuximab vedotin with cyclophosphamide, doxorubicin and prednisone has been approved for patients with untreated aggressive adult T-cell leukemia-lymphoma in Japan. Importantly, the aging of patients with adult T-cell leukemia-lymphoma has recently been reported, and no standard of care for elderly patients with adult T-cell leukemia-lymphoma has been established. New evidence must be obtained from prospective clinical trials to improve the prognosis of patients with adult T-cell leukemia-lymphoma.© The Author(s) 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.