Department of Hematology, Sasebo City General Hospital, Sasebo, Japan.
Department of Hematology and Rheumatology, Kagoshima University, Kagoshima, Japan.
Jpn J Clin Oncol. 2023 Dec 7;53(12):1104-1111. doi: 10.1093/jjco/hyad108.
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.
成人 T 细胞白血病淋巴瘤定义为人类 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惰性（有利的慢性或亚临床）或侵袭性（急性、淋巴瘤或不利的慢性）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以及成人 T 细胞白血病淋巴瘤治疗中存在的未满足需求。对于惰性成人 T 细胞白血病淋巴瘤，建议在疾病进展为侵袭性成人 T 细胞白血病淋巴瘤之前采取观察等待策略。对于侵袭性成人 T 细胞白血病淋巴瘤，建议采用多药化疗联合或不联合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，新型药物，包括 mogamulizumab、lenalidomide、brentuximab vedotin、tucidinostat 和 valemetostat，已被批准用于治疗复发或难治性侵袭性成人 T 细胞白血病淋巴瘤患者，mogamulizumab 联合多药化疗或 brentuximab vedotin 联合环磷酰胺、多柔比星和泼尼松已被批准用于未经治疗的侵袭性成人 T 细胞白血病淋巴瘤患者。重要的是，最近报道了成人 T 细胞白血病淋巴瘤患者的老龄化问题，并且尚未为老年成人 T 细胞白血病淋巴瘤患者建立标准治疗。必须从前瞻性临床试验中获得新的证据，以改善成人 T 细胞白血病淋巴瘤患者的预后。
