在 T 细胞急性淋巴细胞白血病/淋巴瘤 (T-ALL/LBL) 中最佳一线使用活性药物对于改善预后是谨慎的。我们报告了使用奈拉滨和聚乙二醇化天冬酰胺酶进行的 HyperCVAD 2 期试验的长期随访（原始队列）。在最新的方案迭代中，维奈托克被添加到诱导/巩固方案中（维奈托克队列）。符合条件的患者是患有未经治疗的 T-ALL/LBL 或经过最低限度治疗且具有足够器官功能的成人。该分析的主要终点是维奈​​托克治疗后 2 年无进展生存期 (PFS) 和总生存期 (OS) 的改善。从2007年8月到2024年12月，共治疗了145名患者，平均年龄为35.4岁； 46 例 (33.8%) 属于 Venetoclax 队列。中位随访时间 (mFU) 为 62.4 个月，5 年 PFS、缓解持续时间 (DOR) 和 OS 分别为 63.7%、72.0% 和 66.2%。在 Venetoclax 队列（mFU 24.4 个月）中，2 年 PFS（87.9% 对比 64.1%，p = 0.03）和 2 年 DOR（93.6% 对比 69.2%，p = 0.005）优于原始队列（mFU 89.4 个月） ）和 2 年 OS 似乎更好（87.8% 对比 73.9%，p = 0.16）。发热性中性粒细胞减少症是最常见的严重不良事件，发生于 60% 的患者。在 HyperCVAD-奈拉滨-聚乙二醇化天冬酰胺酶中添加 Venetoclax 是可以耐受的，并且改善了 DOR 和 PFS。© 2024。作者，获得 Springer Nature Limited 的独家许可。

Optimal frontline use of active agents in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL/LBL) is prudent to improve outcomes. We report the long-term follow-up of the phase 2 trial of HyperCVAD with nelarabine and pegylated asparaginase (Original cohort). In the latest protocol iteration venetoclax was added to the induction/consolidation regimen (Venetoclax cohort). Eligible patients were adults with untreated T-ALL/LBL or after minimal therapy and with adequate organ function. Primary endpoint of this analysis was improvement in 2-year progression free survival (PFS) and overall survival (OS) with venetoclax. From Aug 2007 to Dec 2024, 145 patients, at a median age of 35.4 years, were treated; 46 (33.8%) were in the venetoclax cohort. At median follow-up (mFU) of 62.4 months, 5-year PFS, duration of response (DOR), and OS were 63.7%, 72.0% and 66.2% respectively. In the venetoclax cohort (mFU 24.4 months) 2-year PFS (87.9% versus 64.1%, p = 0.03) and 2-year DOR (93.6% versus 69.2%, p = 0.005) were superior to the original cohort (mFU 89.4 months) and 2-year OS appeared better (87.8% versus 73.9%, p = 0.16). Febrile neutropenia was the most common serious adverse event, seen in 60% patients. The addition of venetoclax to HyperCVAD-nelarabine-pegylated asparaginase was tolerable and led to improvement in DOR and PFS.© 2024. The Author(s), under exclusive licence to Springer Nature Limited.