幕上细胞淋巴瘤(MCL)是一种目前被视作不可治愈的B细胞恶性肿瘤。尽管一些患者在一线化学免疫治疗后获得了长时间的缓解，但许多患者仍需要进行多线治疗。在这里，我们对MCL的治疗策略、进展时间和生存状况进行了全国范围的评估。在瑞典淋巴瘤登记中心，确定了所有2006年至2018年被诊断为MCL的患者。从病历中提取了关于所有治疗线的信息。通过2021年8月对总体生存和进展无病生存(OS和PFS)进行评估。总计包括1367名患者(中位年龄71岁)，中位随访时间为6.8年。其中201名(15%)最初采用观察与等待的方式进行管理，但最终有1235名(90%)患者接受了治疗。最常用的一线治疗方案是利妥昔单抗-苯丁脢（BR）(n = 368; 30%)和北欧MCL2(n = 342; 28%)。随访期间，630名患者(46%)出现了复发/进展，546名(40%)接受了二线治疗。最常用的二线治疗方案是BR(n = 185; 34%)，但也使用了多种不同的二线治疗方法。此外，382名和228名患者分别经历了第二次或第三次复发/进展。第一(PFS-1)、第二(PFS-2)、第三(PFS-3)和第四(PFS-4)治疗线后的中位无进展生存期分别为29.4、8.9、4.3和2.7个月。早期进展患者，即PFS-1 < 24个月的患者，其中位总生存期较短，为13个月，而后续复发的患者中中位总生存期为37个月。然而，对于采用BR作为一线治疗的患者，复发时间对后续结局没有影响。通过使用全国人口基础数据，我们为MCL在所有治疗线上进行未来研究提供了重要的基准。版权所有© 2023 作者。由 Wolters Kluwer Health, Inc. 代表欧洲血液学会发表。

Mantle cell lymphoma (MCL) is a B-cell malignancy currently considered incurable. Although some patients obtain prolonged remission after first-line chemoimmunotherapy, many will need several treatment lines. Here, we present a nationwide assessment of treatment strategies, time to progression and survival in MCL. All patients diagnosed with MCL 2006-2018 were identified in the Swedish Lymphoma Register. Information on all lines of therapy was extracted from the medical records. Overall and progression-free survival (OS and PFS) were assessed through August 2021. In total, 1367 patients were included (median age, 71 years) and median follow-up was 6.8 years. Two hundred and one (15%) were managed initially with watch-and-wait, but 1235 (90%) eventually received treatment. The most frequently used first-line regimens were rituximab-bendamustine (BR) (n = 368; 30%) and Nordic MCL2 (n = 342; 28%). During follow-up, 630 patients (46%) experienced relapse/progression and 546 (40%) received second-line treatment. The most frequently used second-line regimen was BR (n = 185; 34%) but otherwise a wide variety of second-line treatments were used. Further, 382 and 228 patients experienced a second or third relapse/progression, respectively. Median PFS after first (PFS-1), second (PFS-2), third (PFS-3), and fourth (PFS-4) treatment lines was 29.4, 8.9, 4.3, and 2.7 months. Patients with early progression, defined as a PFS-1 <24 months, had an inferior median OS of 13 versus 37 months in patients with later relapse. For patients treated with frontline BR, however, time to relapse had no impact on later outcome. By use of nationwide population-based data, we provide important benchmarks for future studies of all treatment lines in MCL.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.