Department of Medicine 2, Hematology, Oncology, Hemostaseology, Rheumatology and Infectious Diseases, Goethe University, Theodor-Stern-Kai 7, D-60590 Frankfurt am Main, Germany.
University Hospital Frankfurt, Frankfurt am Main, German Cancer Consortium (DKTK), partner site Frankfurt and DKFZ, D-69120 Heidelberg, Germany.
Biol Chem. 2021 Oct 27;402(12):1547-1564. doi: 10.1515/hsz-2021-0288. Print 2021 Nov 25.
Acute myeloid leukemia (AML) is a heterogeneous, highly malignant disease of the bone marrow. After decades of slow progress, recent years saw a surge of novel agents for its treatment. The most recent advancement is the registration of the Bcl-2 inhibitor ventoclax in combination with a hypomethylating agent (HMA) in the US and Europe for AML patients not eligible for intensive chemotherapy. Treatment of newly diagnosed AML patients with this combination results in remission rates that so far could only be achieved with intensive treatment. However, not all AML patients respond equally well, and some patients relapse early, while other patients experience longer periods of complete remission. A hallmark of AML is its remarkable genetic, molecular and clinical heterogeneity. Here, we review the current knowledge about molecular features of AML that help estimate the probability of response to venetoclax-containing therapies. In contrast to other newly developed AML therapies that target specific recurrent molecular alterations, it seems so far that responses are not specific for a certain subgroup. One exception is spliceosome mutations, where good response has been observed in clinical trials with venetoclax/azacitidine. These mutations are rather associated with a more unfavorable outcome with chemotherapy. In summary, venetoclax in combination with hypomethylating agents represents a significant novel option for AML patients with various molecular aberrations. Mechanisms of primary and secondary resistance seem to overlap with those towards chemotherapy.
急性髓细胞白血病(AML)是一种异质性、高度恶性的骨髓疾病。经过几十年的缓慢进展,近年来出现了许多新型药物来治疗 AML。最近的进展是,Bcl-2 抑制剂 venetoclax 联合低甲基化剂(HMA)在美国和欧洲注册,用于不适合强化化疗的 AML 患者。这种联合疗法治疗新诊断的 AML 患者的缓解率迄今为止只能通过强化治疗来实现。然而,并非所有 AML 患者的反应都一样,一些患者早期复发,而另一些患者则经历更长时间的完全缓解。AML 的一个显著特征是其显著的遗传、分子和临床异质性。在这里,我们回顾了目前关于 AML 分子特征的知识,这些知识有助于估计对含 venetoclax 疗法的反应概率。与其他针对特定复发性分子改变的新开发的 AML 疗法不同,到目前为止,似乎反应并不是针对特定亚组的。一个例外是剪接体突变,在 venetoclax/阿扎胞苷的临床试验中观察到了良好的反应。这些突变与化疗的预后更差有关。总之,venetoclax 联合低甲基化剂为具有各种分子异常的 AML 患者提供了一种重要的新选择。原发性和继发性耐药的机制似乎与化疗的机制重叠。