中国全科医学 ›› 2026, Vol. 29 ›› Issue (04): 483-489.DOI: 10.12114/j.issn.1007-9572.2025.0270

• 论著 • 上一篇    下一篇

组蛋白去乙酰化酶抑制剂联合维奈克拉和阿扎胞苷治疗成人急性T淋巴细胞白血病的疗效及安全性研究

付积艺1, 郑博月1, 吴佳霏1, 王珺2, 李慧1,3,*()   

  1. 1.610056 四川省成都市,电子科技大学医学院
    2.610056 四川省成都市第二人民医院血液科
    3.610056 四川省成都市,四川省医学科学院·四川省人民医院 电子科技大学附属医院血液科
  • 收稿日期:2025-07-09 修回日期:2025-10-27 出版日期:2026-02-05 发布日期:2026-01-15
  • 通讯作者: 李慧

  • 作者贡献:

    付积艺、郑博月、吴佳霏、王珺负责数据收集;付积艺、郑博月负责数据分析;付积艺负责论文初稿撰写;郑博月、吴佳霏、王珺负责患者随访;李慧主导研究课题设计,审阅并修订文章,对研究完整性承担最终责任。

  • 基金资助:
    四川省自然科学基金资助项目(23NSFSC0607); 国家卫生健康委医药卫生科技发展研究中心资助项目(WKZX2024CX105201)

Efficacy and Safety of a Combined Regimen of Histone Deacetylase Inhibitors Venetoclax and Azacitidine in Adult T-cell Acute Lymphoblastic Leukemia

FU Jiyi1, ZHENG Boyue1, WU Jiafei1, WANG Jun2, LI Hui1,3,*()   

  1. 1. School of Medicine, University of Electronic Science and Technology, Chengdu 610056, China
    2. Hematology Department, Chengdu Second People's Hospital, Chengdu 610056, China
    3. Department of Hematology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital/Affiliated Hospital of UESTC, Chengdu 610056, China
  • Received:2025-07-09 Revised:2025-10-27 Published:2026-02-05 Online:2026-01-15
  • Contact: LI Hui

摘要: 背景 急性T淋巴细胞白血病(T-ALL)易产生原发耐药、诱导失败、中枢浸润、早期复发等,目前传统化疗方案缓解率低、复发率高、不良反应发生率高,成人T-ALL患者总体预后差、远期生存率低。目的 采用靶向表观遗传通路的联合方案[DNA甲基转移酶抑制剂阿扎胞苷+组蛋白去乙酰化酶(HDAC)抑制剂]与凋亡通路抑制剂维奈克拉,通过多靶点协同作用治疗T-ALL患者,评估HDAC抑制剂联合维奈克拉及阿扎胞苷在T-ALL患者中的疗效与安全性。方法 本研究是一项探索性、单中心、单臂研究,纳入2023年6月—2025年1月四川省人民医院血液科收治的12例T-ALL患者为研究对象,收集患者的基线资料(临床特征及分子遗传学特征),患者均接受HDAC抑制剂、阿扎胞苷、维奈克拉及地塞米松联合治疗,每半月为1个疗程。治疗1~2个疗程后进行疗效评估,并通过门诊复查、住院复查、电话随访或病历系统随访完成患者随访。根据患者白血病分型分为早期前体T淋巴母细胞白血病(ETP)组、非早期前体T淋巴母细胞白血病(non-ETP)组,记录完全缓解(CR)率、总反应率(ORR)、微小残留病(MRD)阴性率、总生存率(OS)、无事件生存期(EFS),并进行亚组间分析;采用Kaplan-Meier法绘制患者生存曲线,Log-rank检验进行比较。采用单因素Cox回归分析探讨T-ALL患者预后相关因素。记录患者不良事件发生情况。结果 治疗1个疗程后,12例患者中ORR为83.3%(10/12),其中CR为5例(41.7%),MRD阴性为2例(16.7%)。治疗2个疗程后,12例患者中ORR为91.7%(11/12),MRD阴性为8例(66.7%)。7例患者在接受2或3个疗程化疗后成功桥接骨髓移植,移植后目前患者全部存活。3例患者因自行停药死亡。中位随访时间为6.5(2.0,22.0)个月,中位OS及中位EFS均未达到。ETP组、non-ETP组临床疗效相比差异无统计学意义(P>0.05)。Log-rank检验结果显示ETP组预后较non-ETP组更差(χ2=4.830,P=0.028);移植患者预后较未移植患者更好(χ2=6.545,P=0.011)。2个疗程治疗后CR患者预后较未达CR患者更好(χ2=4.571,P=0.033);MRD阴性患者预后较未达MRD阴性患者更好(χ2=4.571,P=0.033)。骨髓原始细胞占比>50%与<50%患者预后情况比较,差异无统计学意义(χ2=0.171,P=0.67)。单因素Cox分析结果显示,性别、年龄、T-ALL类型、突变基因数量、染色体核型、疗效、MRD、移植均不是患者OS的影响因素(P>0.05)。患者均发生至少1起不良事件,较常报道的≥3级血液学不良事件包括中性粒细胞减少(50.0%)、血小板减少(41.7%)和贫血(25.0%);肝功能损害、肾功能损害发生率较低,分别为16.7%、8.3%;胃肠道不良事件主要是恶心呕吐(83.3%);共有5例患者发生肺炎(41.7%),其中ETP组的1例患者发生≥3级肺炎及≥3级脓毒血症;未报道心血管系统不良事件。结论 HDAC抑制剂联合维奈克拉和阿扎胞苷方案对T-ALL患者是一种有效的诱导治疗策略,可实现更高和更深的缓解,且具有良好的安全性和耐受性,为进一步行异基因造血干细胞移植争取最大机会,值得进一步开展大样本研究验证。

关键词: 白血病, T细胞, 组蛋白去乙酰化酶抑制剂, 阿扎胞苷, 维奈克拉, 联合用药, 预后

Abstract:

Background

Adult T-cell acute lymphoblastic leukemia (T-ALL) is often associated with primary resistance, induction failure, central nervous system infiltration, and early relapse. Traditional chemotherapy regimens have shown low remission rates, high recurrence rates, and high incidence of adverse events, leading to poor overall prognosis and low long-term survival rates for adult T-ALL patients.

Objective

To evaluate the efficacy and safety of a targeted epigenetic pathway regimen [DNA methyltransferase inhibitor azacitidine + histone deacetylase (HDAC) inhibitor)] combined with an apoptosis pathway inhibitor, venetoclax, in treating T-ALL patients through multi-target synergistic effects.

Methods

This exploratory, single-center, single-arm study included 12 T-ALL patients admitted to the Hematology Department of Sichuan Provincial People's Hospital from June 2023 to January 2025. Baseline data (clinical features and molecular genetic characteristics) were collected. All patients received a combination therapy of HDAC inhibitors, azacitidine, venetoclax, and dexamethasone, with each cycle lasting half a month. Efficacy was assessed after 1-2 cycles of treatment. Follow-up was conducted via outpatient review, inpatient review, telephone follow-up, or medical record system follow-up. Patients were categorized into early T-cell precursor ALL (ETP) and non-early T-cell precursor ALL (non-ETP) groups, recording complete response (CR) rate, overall response rate (ORR), minimal residual disease (MRD) negativity rate, overall survival (OS), and event-free survival (EFS). Subgroup analyses were performed using Kaplan-Meier curves and Log-rank tests for survival comparison and univariate Cox regression analysis for prognostic factors exploration. Adverse events were also recorded.

Results

After one cycle of treatment, ORR among 12 patients was 83.3% (10/12), with CR in 5 cases (41.7%). After two cycles, ORR increased to 91.7% (11/12), and MRD negativity rose to 8 cases (66.7%). Seven patients successfully bridged to bone marrow transplantation post 2-3 cycles of chemotherapy, all currently surviving. Three deaths occurred due to self-discontinuation of medication. The median follow-up time was 6.5 months, without reaching median OS or EFS. There was no significant difference in clinical outcomes between ETP and non-ETP groups (P>0.05). However, ETP group showed worse prognosis compared to non-ETP (χ2=4.830, P=0.028), while transplanted patients had better prognosis than non-transplanted ones (χ2=6.545, P=0.011). Moreover, CR patients post two cycles had better prognosis than those not achieving CR (χ2=4.571, P=0.033), as did MRD negative patients versus MRD positive ones (χ2=4.571, P=0.033). Univariate Cox regression analysis indicated that gender, age, T-ALL subtype, number of mutated genes, chromosomal karyotype, efficacy, MRD status, and transplantation were not significant predictors of OS (P>0.05). All patients experienced at least one adverse event, with common grade ≥3 hematological adverse events including neutropenia (50.0%), thrombocytopenia (41.7%), and anemia (25.0%). Hepatotoxicity and nephrotoxicity were less frequent at 16.7% and 8.3%, respectively. Gastrointestinal adverse events primarily consisted of nausea and vomiting (83.3%), with pneumonia occurring in 5 patients (41.7%).

Conclusion

The combination of HDAC inhibitors, venetoclax, and azacitidine represents an effective induction therapy strategy for adult T-ALL, achieving higher and deeper remissions with acceptable safety profiles, making it a promising approach worthy of further investigation in larger studies.

Key words: Leukemia, T-cell, Histone deacetylase inhibitor, Azacytidine, Venetoclax, Combined medication, Prognosis