Mihalyova Jana, Jelinek Tomas, Growkova Katerina, Hrdinka Matous, Simicek Michal, Hajek Roman
Department of Haematooncology, University Hospital Ostrava, Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic.
Department of Haematooncology, University Hospital Ostrava, Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic; Faculty of Science, University of Ostrava, Ostrava, Czech Republic.
Exp Hematol. 2018 May;61:10-25. doi: 10.1016/j.exphem.2018.02.002. Epub 2018 Mar 2.
Inhibitors of antiapoptotic proteins of the BCL2 family can successfully restart the deregulated process of apoptosis in malignant cells. Whereas nonselective agents have been limited by their affinity to different BCL2 members, thus inducing excessive toxicity, the highly selective BCL2 inhibitor venetoclax (ABT-199, Venclexta™) has an acceptable safety profile. To date, it has been approved in monotherapy for the treatment of relapsed or refractory chronic lymphocytic leukemia (CLL) with 17p deletion. Extension of indications can be expected in monotherapy and in combination regimens. Sensitivity to venetoclax is not common in lymphomas, but promising outcomes have been achieved in the mantle cell lymphoma group. Venetoclax is also active in multiple myeloma patients, especially in those with translocation t(11;14), even if high-risk features such as del17p are also present. Surprisingly, positive results are being obtained in elderly acute myeloid leukemia patients, in whom inhibition of BCL2 is able to substantially increase the efficacy of low-dose cytarabine or hypomethylating agents. Here, we provide a summary of available results from clinical trials and describe a specific mechanism of action that stands behind the efficacy of venetoclax in hematological malignancies.
BCL2家族抗凋亡蛋白抑制剂能够成功重启恶性细胞中失调的凋亡过程。非选择性药物由于对不同BCL2成员的亲和力而受到限制,从而导致过度毒性,而高选择性的BCL2抑制剂维奈克拉(ABT - 199,Venclexta™)具有可接受的安全性。迄今为止,它已被批准用于单药治疗伴有17p缺失的复发或难治性慢性淋巴细胞白血病(CLL)。预计其单药治疗及联合治疗方案的适应证将会扩大。维奈克拉在淋巴瘤中并不常见,但在套细胞淋巴瘤组已取得了有前景的结果。维奈克拉在多发性骨髓瘤患者中也有活性,尤其是那些存在t(11;14)易位的患者,即使同时存在如del17p等高危特征。令人惊讶的是,老年急性髓系白血病患者也获得了阳性结果,在这些患者中,抑制BCL2能够显著提高低剂量阿糖胞苷或去甲基化药物的疗效。在此,我们总结了临床试验的现有结果,并描述了维奈克拉在血液系统恶性肿瘤中发挥疗效背后的具体作用机制。