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东京治疗成人T细胞白血病/淋巴瘤的核心医院中70岁及以下侵袭性成人T细胞白血病/淋巴瘤患者的治疗结果。

Outcomes of patients aged 70 years or younger with aggressive ATL at core hospitals for ATL treatment in Tokyo.

作者信息

Makiyama Junya, Ohno Nobuhiro, Jimbo Koji, Kawamata Toyotaka, Yokoyama Kazuaki, Konuma Takaaki, Kato Seiko, Takemura Tomonari, Ito Ayumu, Tanaka Takashi, Inamoto Yoshihiro, Fuji Shigeo, Imai Yoichi, Takahashi Satoshi, Nannya Yasuhito, Tojo Arinobu, Fukuda Takahiro, Uchimaru Kaoru

机构信息

Department of Hematology/Oncology, Research Hospital, The Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, Japan.

Department of Hematology, Sasebo City General Hospital, Nagasaki, Japan.

出版信息

Int J Hematol. 2025 Sep 2. doi: 10.1007/s12185-025-04057-2.

Abstract

Adult T-cell leukemia-lymphoma (ATL) is one of the most intractable peripheral T-cell neoplasms caused by human T-cell leukemia virus type I (HTLV-1) infection. Recently, the incidence of HTLV-1 infection and ATL has increased in non-endemic metropolitan areas in Japan. This retrospective study evaluated the clinical features and outcomes of patients with aggressive ATL aged 70 years or younger treated at a core hospital in Tokyo between 2004 and 2016. The median follow-up was 124.4 months for survivors. Among the 71 patients, 46 (64.8%) underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT). The 3 year overall survival rate was 45.7% in allo-HSCT group versus 0% in non-allo-HSCT group. Patients who achieved complete/partial remission before allo-HSCT had a significantly better survival rate than those with stable/progressive disease (51.4% vs 27.3%). The 2 year cumulative incidence of relapse/progression and non-relapse mortality after allo-HSCT was 41.3% and 21.7%, respectively. In this study, a large percentage of patients underwent allo-HSCT and achieved favorable outcomes. As cases continue to rise in metropolitan areas, core hospitals will play a critical role in ATL treatment.

摘要

成人T细胞白血病-淋巴瘤(ATL)是由I型人类T细胞白血病病毒(HTLV-1)感染引起的最难治疗的外周T细胞肿瘤之一。最近,在日本的非流行大都市地区,HTLV-1感染和ATL的发病率有所上升。这项回顾性研究评估了2004年至2016年期间在东京一家核心医院接受治疗的70岁及以下侵袭性ATL患者的临床特征和预后。幸存者的中位随访时间为124.4个月。在71例患者中,46例(64.8%)接受了异基因造血干细胞移植(allo-HSCT)。allo-HSCT组的3年总生存率为45.7%,而非allo-HSCT组为0%。在allo-HSCT前达到完全/部分缓解的患者的生存率明显高于疾病稳定/进展的患者(51.4%对27.3%)。allo-HSCT后复发/进展和非复发死亡率的2年累积发生率分别为41.3%和21.7%。在本研究中,很大比例的患者接受了allo-HSCT并取得了良好的结果。随着大都市地区病例的持续增加,核心医院将在ATL治疗中发挥关键作用。

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