Division of Hematology/Oncology, Department of Medicine, Hospital of the University of Pennsylvania, South Pavilion, 12th Floor, 3400 Civic Center Blvd, Philadelphia, PA, 19104, USA.
J Hematol Oncol. 2023 Mar 25;16(1):29. doi: 10.1186/s13045-023-01424-6.
Acute myeloid leukemia (AML) is the most common acute leukemia in adults. While survival for younger patients over the last several decades has improved nearly sixfold with the optimization of intensive induction chemotherapy and allogeneic stem cell transplantation (alloHSCT), this effect has been largely mitigated in older and less fit patients as well as those with adverse-risk disease characteristics. However, the last 10 years has been marked by major advances in the molecular profiling of AML characterized by a deeper understanding of disease pathobiology and therapeutic vulnerabilities. In this regard, the classification of AML subtypes has recently evolved from a morphologic to a molecular and genetic basis, reflected by recent updates from the World Health Organization and the new International Consensus Classification system. After years of stagnation in new drug approvals for AML, there has been a rapid expansion of the armamentarium against this disease since 2017. Low-intensity induction therapy with hypomethylating agents and venetoclax has substantially improved outcomes, including in those previously considered to have a poor prognosis. Furthermore, targeted oral therapies against driver mutations in AML have been added to the repertoire. But with an accelerated increase in treatment options, several questions arise such as how to best sequence therapy, how to combine therapies, and if there is a role for maintenance therapy in those who achieve remission and cannot undergo alloHSCT. Moreover, certain subtypes of AML, such as those with TP53 mutations, still have dismal outcomes despite these recent advances, underscoring an ongoing unmet need and opportunity for translational advances. In this review, we will discuss recent updates in the classification and risk stratification of AML, explore the literature regarding low-intensity and novel oral combination therapies, and briefly highlight investigative agents currently in early clinical development for high-risk disease subtypes.
急性髓系白血病 (AML) 是成人中最常见的急性白血病。在过去几十年中,通过优化强化诱导化疗和异基因干细胞移植 (alloHSCT),年轻患者的生存率提高了近六倍,但这一效果在年龄较大、身体状况较差的患者以及患有不良风险疾病特征的患者中已大大减轻。然而,在过去的 10 年中,AML 的分子谱分析取得了重大进展,这使得人们对疾病的病理生物学和治疗弱点有了更深入的了解。在这方面,AML 亚型的分类最近已经从形态学发展到基于分子和遗传的基础,这反映在世界卫生组织和新的国际共识分类系统的最新更新中。在 AML 新药批准多年停滞不前之后,自 2017 年以来,针对这种疾病的武器库迅速扩大。低强度诱导化疗联合低甲基化剂和 venetoclax 已显著改善了包括以前被认为预后不良的患者在内的预后。此外,针对 AML 驱动突变的靶向口服疗法已被添加到方案中。但是,随着治疗选择的加速增加,出现了一些问题,例如如何最佳地进行治疗顺序、如何联合治疗以及对于那些达到缓解但不能进行 alloHSCT 的患者是否有维持治疗的作用。此外,尽管最近取得了这些进展,但某些 AML 亚型,如那些具有 TP53 突变的亚型,其预后仍然不佳,这突显了仍存在未满足的需求和转化进展的机会。在这篇综述中,我们将讨论 AML 的分类和风险分层的最新更新,探讨关于低强度和新型口服联合治疗的文献,并简要介绍目前处于早期临床开发阶段的高风险疾病亚型的研究性药物。