Garciaz Sylvain, Montersino Camille, Bourgoin Maxence, Jacquel Arnaud, Castellano Rémy, Guille Arnaud, Chaffanet Max, Bertucci François, Hospital Marie-Anne, Adélaïde José, Auberger Patrick, Vey Norbert, Collette Yves
Aix-Marseille Univ, Inserm, CNRS, Institut Paoli-Calmettes, CRCM, Marseille, France.
INSERM U1065 Centre Méditerranéen de Médecine Moléculaire, Nice, France.
Br J Haematol. 2025 Jun 6;207(2):381-6. doi: 10.1111/bjh.20195.
Acute myeloid leukaemia (AML) is a severe disease occurring mainly in the elderly population. Venetoclax (VEN) combined with azacitidine has changed the paradigm of treatment of AML. Nevertheless, approximately 30% of patients are primary refractory to VEN (VEN-R), with no current therapeutic option. To target VEN-R AML, we collected primary blasts at AML diagnosis in a prospective biobanking trial (NCT02320656). We performed targeted Next Generation Sequencing and ex vivo drug testing in 108 AML samples. We noticed that 17 (15.7%) were navitoclax-resistant (NAV-R). We observed a strong anticorrelation between NAV and Dasatinib (DASA) ex vivo sensitivity, also found in the BEAT-AML cohort. As NAV and ABT797 are both BCL2/BCLxL inhibitors, we hypothesized that blasts sensitive to DASA (DASA-S) were dependent on MCL1. We performed BH3 profiling in 25 samples confirming MCL1 dependency. Immunoblots showed a higher MCL1 and BIM protein expression. We found a dose-dependent decrease in MCL1 protein expression associated with caspase 3 activation upon DASA in a primary AML sample. Collectively, these results suggest that DASA degrades MCL1 and effectively kills AML cells. To prove this hypothesis, we designed a phase II clinical trial named VEN-R DASA-IPC 2022 067 (EUCT 2023-505846-24-00), currently enrolling VEN-R patients.
急性髓系白血病(AML)是一种主要发生在老年人群中的严重疾病。维奈克拉(VEN)联合阿扎胞苷改变了AML的治疗模式。然而,约30%的患者对VEN原发性难治(VEN-R),目前尚无治疗选择。为了针对VEN-R AML,我们在一项前瞻性生物样本库试验(NCT02320656)中收集了AML诊断时的原发性原始细胞。我们对108个AML样本进行了靶向二代测序和体外药物测试。我们注意到17个样本(15.7%)对纳维托克耐药(NAV-R)。我们观察到NAV与达沙替尼(DASA)体外敏感性之间存在强烈的负相关,这在BEAT-AML队列中也有发现。由于NAV和ABT797都是BCL2/BCLxL抑制剂,我们推测对DASA敏感的原始细胞(DASA-S)依赖于MCL1。我们在25个样本中进行了BH3分析,证实了对MCL1的依赖性。免疫印迹显示MCL1和BIM蛋白表达更高。我们在一个原发性AML样本中发现,DASA作用后,MCL1蛋白表达呈剂量依赖性下降,并伴有caspase 3激活。总体而言,这些结果表明DASA可降解MCL1并有效杀死AML细胞。为了验证这一假设,我们设计了一项名为VEN-R DASA-IPC 2022 067(EUCT 2023-505846-24-00)的II期临床试验,目前正在招募VEN-R患者。