Guignabert Christophe, Phan Carole, Seferian Andrei, Huertas Alice, Tu Ly, Thuillet Raphaël, Sattler Caroline, Le Hiress Morane, Tamura Yuichi, Jutant Etienne-Marie, Chaumais Marie-Camille, Bouchet Stéphane, Manéglier Benjamin, Molimard Mathieu, Rousselot Philippe, Sitbon Olivier, Simonneau Gérald, Montani David, Humbert Marc
J Clin Invest. 2016 Sep 1;126(9):3207-18. doi: 10.1172/JCI86249. Epub 2016 Aug 2.
Pulmonary arterial hypertension (PAH) is a life-threatening disease that can be induced by dasatinib, a dual Src and BCR-ABL tyrosine kinase inhibitor that is used to treat chronic myelogenous leukemia (CML). Today, key questions remain regarding the mechanisms involved in the long-term development of dasatinib-induced PAH. Here, we demonstrated that chronic dasatinib therapy causes pulmonary endothelial damage in humans and rodents. We found that dasatinib treatment attenuated hypoxic pulmonary vasoconstriction responses and increased susceptibility to experimental pulmonary hypertension (PH) in rats, but these effects were absent in rats treated with imatinib, another BCR-ABL tyrosine kinase inhibitor. Furthermore, dasatinib treatment induced pulmonary endothelial cell apoptosis in a dose-dependent manner, while imatinib did not. Dasatinib treatment mediated endothelial cell dysfunction via increased production of ROS that was independent of Src family kinases. Consistent with these findings, we observed elevations in markers of endothelial dysfunction and vascular damage in the serum of CML patients who were treated with dasatinib, compared with CML patients treated with imatinib. Taken together, our findings indicate that dasatinib causes pulmonary vascular damage, induction of ER stress, and mitochondrial ROS production, which leads to increased susceptibility to PH development.
肺动脉高压(PAH)是一种危及生命的疾病,可由达沙替尼诱发,达沙替尼是一种用于治疗慢性粒细胞白血病(CML)的双靶点Src和BCR-ABL酪氨酸激酶抑制剂。如今,关于达沙替尼诱发PAH的长期发展机制仍存在关键问题。在此,我们证明慢性达沙替尼治疗会导致人类和啮齿动物的肺内皮损伤。我们发现,达沙替尼治疗减弱了大鼠的低氧性肺血管收缩反应,并增加了其对实验性肺动脉高压(PH)的易感性,但在接受另一种BCR-ABL酪氨酸激酶抑制剂伊马替尼治疗的大鼠中未观察到这些效应。此外,达沙替尼治疗以剂量依赖的方式诱导肺内皮细胞凋亡,而伊马替尼则不会。达沙替尼治疗通过增加ROS的产生介导内皮细胞功能障碍,这一过程独立于Src家族激酶。与这些发现一致,我们观察到达沙替尼治疗的CML患者血清中内皮功能障碍和血管损伤标志物水平升高,而接受伊马替尼治疗的CML患者则无此现象。综上所述,我们的研究结果表明,达沙替尼会导致肺血管损伤、内质网应激诱导和线粒体ROS产生,从而增加对PH发展的易感性。