Bruzzese Antonella, Vigna Ernesto, Martino Enrica Antonia, Labanca Caterina, Caridà Giulio, Mendicino Francesco, Lucia Eugenio, Olivito Virginia, Puccio Noemi, Neri Antonino, Morabito Fortunato, Gentile Massimo
Hematology Unit, Department of Onco-Hematology, AO of Cosenza, Cosenza, Italy.
Department of Experimental and Clinical Medicine, University of Catanzaro, Catanzaro, Italy.
Hematol Oncol. 2025 Jul;43(4):e70106. doi: 10.1002/hon.70106.
TP53-mutated acute myeloid leukemia (AML) remains one of the most treatment-resistant hematologic malignancies, with poor overall survival despite advancements in therapeutic strategies. The loss of functional p53 compromises DNA repair, apoptosis, and genomic stability, rendering both conventional and novel therapies largely ineffective. This review evaluates the efficacy of various treatment approaches, including intensive chemotherapy (IC), hypomethylating agents (HMAs), venetoclax-based regimens, and immune checkpoint inhibitors. Additionally, we discuss emerging strategies such as p53 reactivation, multi-targeted inhibition, and novel immunotherapies, including bispecific T-cell engagers (BiTEs) and CAR-T cell therapy. Current treatment options provide limited benefits in TP53-mutated AML, with complete remission rates ranging from 13% to 46% and median overall survival of only 6.1-6.5 months. Allogeneic stem cell transplantation (allo-SCT) offers minimal survival advantage due to high relapse rates. Despite promising preclinical data, checkpoint inhibitors and TIM-3 blockade have failed to demonstrate significant clinical efficacy, likely due to the immunosuppressive tumor microenvironment. Novel approaches, such as APR-246 (eprenetapopt) and MCL-1/CHK1 inhibitors, are under investigation, but their therapeutic impact remains uncertain. The failure of single-agent therapies underscores the need for combination strategies targeting multiple resistance mechanisms. Future research should focus on integrating targeted inhibitors with immunotherapy and bone marrow microenvironment modifiers. While TP53-mutated AML remains a formidable challenge, ongoing advances in precision medicine and immunotherapy hold the potential to improve patient outcomes.
TP53 突变的急性髓系白血病(AML)仍然是最难治疗的血液系统恶性肿瘤之一,尽管治疗策略有所进步,但总体生存率仍很低。功能性 p53 的缺失会损害 DNA 修复、细胞凋亡和基因组稳定性,使得传统疗法和新型疗法大多无效。本综述评估了包括强化化疗(IC)、去甲基化药物(HMAs)、基于维奈克拉的方案以及免疫检查点抑制剂在内的各种治疗方法的疗效。此外,我们还讨论了诸如 p53 重新激活、多靶点抑制以及新型免疫疗法(包括双特异性 T 细胞衔接器(BiTEs)和嵌合抗原受体 T 细胞(CAR-T)疗法)等新兴策略。目前的治疗选择在 TP53 突变的 AML 中获益有限,完全缓解率在 13%至 46%之间,中位总生存期仅为 6.1 - 6.5 个月。异基因干细胞移植(allo-SCT)由于高复发率,生存优势极小。尽管临床前数据很有前景,但检查点抑制剂和 TIM-3 阻断未能显示出显著的临床疗效,这可能是由于免疫抑制性肿瘤微环境所致。诸如 APR-246(依普奈达泊)和 MCL-1/CHK1 抑制剂等新型方法正在研究中,但其治疗效果仍不确定。单药疗法的失败凸显了针对多种耐药机制的联合策略的必要性。未来的研究应专注于将靶向抑制剂与免疫疗法以及骨髓微环境调节剂相结合。虽然 TP53 突变的 AML 仍然是一个巨大的挑战,但精准医学和免疫疗法的不断进步有望改善患者的预后。