Department of Medical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing Key Laboratory of Clinical Study on Anticancer Molecular Targeted Drugs, No. 17 Panjiayuan Nanli, Chaoyang District, Beijing, 100021, China.
Ann Hematol. 2020 Dec;99(12):2847-2857. doi: 10.1007/s00277-020-04195-z. Epub 2020 Jul 25.
T cell lymphoblastic lymphoma (T-LBL) has an aggressive clinical behavior. To date, powerful and consistent prognostic factors have not been established for T-LBL. In this study, we first evaluated the association of event-free survival (EFS) at 24 months (EFS24) with overall survival (OS) in T-LBL patients. Besides, we sought to identify clinical factors of prognostic importance in this rare entity. Between January 2006 and December 2017, ninety-one patients with newly diagnosed T-LBL were retrospectively analyzed. EFS was defined as the time from diagnosis to relapse or progression, unplanned retreatment, death from any cause, or to the last follow-up. In total, 91 patients with a median age of 24 years were enrolled. At a median follow-up of 40.4 months (range, 1.4 to 163.3 months), the 5-year OS and EFS was 47.9% and 43.2%, respectively. Of all patients, 45 (49.5%) achieved EFS24 and 46 (50.5%) did not. Patients who achieved EFS24 showed a markedly superior outcome, compared with those who failed to achieve EFS24 (5-year OS, 90.5% vs 3%, P < 0.001). Univariate analysis indicated bone marrow involvement, response to induction treatment, and stem cell transplantation (SCT) consolidation to be prognostic factors for EFS and OS. In addition, compared with the patients receiving non-Hodgkin's lymphoma (NHL)-like treatment protocols, patients treated with hyper-CVAD showed significantly improved EFS and OS. Such survival advantage in terms of EFS and OS was also observed of BMF-90 regimens over NHL-like therapy, despite that the difference in EFS did not reach statistical significance (P = 0.056). Multivariate analysis demonstrated that achievement of complete remission (CR) after induction therapy and SCT consolidation were independent prognostic indicators for both EFS and OS. We confirm that EFS24 is a strong surrogate endpoint for long-term survival in T-LBL, which is clinically useful for individualized risk reassessment, future clinical trial design, and biomarker discovery validation. Further validation in the context of directed prospective clinical trials is warranted.
T 细胞淋巴母细胞淋巴瘤(T-LBL)具有侵袭性的临床行为。迄今为止,尚未为 T-LBL 建立强大且一致的预后因素。在这项研究中,我们首先评估了 24 个月时无事件生存(EFS)与 T-LBL 患者总生存(OS)的相关性。此外,我们试图确定该罕见实体中具有重要预后意义的临床因素。2006 年 1 月至 2017 年 12 月,回顾性分析了 91 例新诊断为 T-LBL 的患者。EFS 定义为从诊断到复发或进展、计划外再治疗、任何原因死亡或最后一次随访的时间。共纳入 91 例中位年龄为 24 岁的患者。中位随访 40.4 个月(范围 1.4 至 163.3 个月),5 年 OS 和 EFS 分别为 47.9%和 43.2%。所有患者中,45 例(49.5%)达到 EFS24,46 例(50.5%)未达到。达到 EFS24 的患者的结局明显优于未达到 EFS24 的患者(5 年 OS,90.5%对 3%,P<0.001)。单因素分析表明骨髓受累、诱导治疗反应和干细胞移植(SCT)巩固是 EFS 和 OS 的预后因素。此外,与接受非霍奇金淋巴瘤(NHL)样治疗方案的患者相比,接受 Hyper-CVAD 治疗的患者的 EFS 和 OS 显著改善。尽管 EFS 差异无统计学意义(P=0.056),但 BMF-90 方案在 EFS 和 OS 方面的生存优势也超过了 NHL 样治疗。多因素分析表明,诱导治疗后获得完全缓解(CR)和 SCT 巩固是 EFS 和 OS 的独立预后指标。我们证实,EFS24 是 T-LBL 长期生存的强有力替代终点,对个体风险重新评估、未来临床试验设计和生物标志物发现验证具有临床意义。需要在定向前瞻性临床试验的背景下进一步验证。