Department of Medical Oncology and Hematology, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy.
School of Medicine, Università degli Studi di Milano, Via Venezian 1, 20133 Milan, Italy.
Int J Mol Sci. 2022 Mar 30;23(7):3834. doi: 10.3390/ijms23073834.
Mature T-cell lymphomas (MTCLs) represent a heterogeneous group of aggressive non-Hodgkin lymphomas comprising different entities. Anthracycline-based regimens are considered the standard of care in the front-line treatment. However, responses to these approaches have been neither adequate nor durable, and new treatment strategies are urgently needed to improve survival. Genomic instability is a common feature of cancer cells and can be caused by aberrations in the DNA damage response (DDR) and DNA repair mechanisms. Consistently, molecules involved in DDR are being targeted to successfully sensitize cancer cells to chemotherapy. Recent studies showed that some hematological malignancies display constitutive DNA damage and intrinsic DDR activation, but these features have not been investigated yet in MTCLs. In this study, we employed a panel of malignant T cell lines, and we report for the first time the characterization of intrinsic DNA damage and basal DDR activation in preclinical models in T-cell lymphoma. Moreover, we report the efficacy of targeting the apical kinase ATM using the inhibitor AZD0156, in combination with standard chemotherapy to promote apoptotic cell death. These findings suggest that DDR is an attractive pathway to be pharmacologically targeted when developing novel therapies and improving MTCL patients' outcomes.
成熟 T 细胞淋巴瘤(MTCLs)是一组异质性的侵袭性非霍奇金淋巴瘤,包含不同的实体。基于蒽环类药物的方案被认为是一线治疗的标准治疗方法。然而,这些方法的反应既不充分也不持久,迫切需要新的治疗策略来提高生存率。基因组不稳定性是癌细胞的一个常见特征,可能是由 DNA 损伤反应(DDR)和 DNA 修复机制的异常引起的。一致地,参与 DDR 的分子被靶向以成功地使癌细胞对化疗敏感。最近的研究表明,一些血液恶性肿瘤表现出固有 DNA 损伤和内在 DDR 激活,但这些特征尚未在 MTCLs 中进行研究。在这项研究中,我们使用了一组恶性 T 细胞系,并首次报告了在 T 细胞淋巴瘤的临床前模型中内在 DNA 损伤和基础 DDR 激活的特征。此外,我们报告了使用抑制剂 AZD0156 靶向顶端激酶 ATM 的疗效,与标准化疗联合使用可促进细胞凋亡。这些发现表明,DDR 是在开发新疗法和改善 MTCL 患者预后时进行药理靶向的一个有吸引力的途径。