University of Pennsylvania, Philadelphia, PA, USA.
Fox Chase Cancer Center, Philadelphia, PA, USA.
Br J Haematol. 2021 Oct;195(2):201-209. doi: 10.1111/bjh.17730. Epub 2021 Aug 2.
Fimepinostat (CUDC-907), a first-in-class oral small-molecule inhibitor of histone deacetylase and phosphatidylinositol 3-kinase, demonstrated efficacy in a phase 1 study of patients with relapsed/refractory (R/R) diffuse large and high-grade B-cell lymphomas (DLBCL/HGBL), particularly those with increased MYC protein expression and/or MYC gene rearrangement/copy number gain (MYC-altered disease). Therefore, a phase 2 study of fimepinostat was conducted in this patient population with 66 eligible patients treated. The primary end-point of overall response (OR) rate for patients with MYC-IHC ≥40% (n = 46) was 15%. Subsequently, exploratory pooled analyses were performed including patients treated on both the phase 1 and 2 studies based upon the presence of MYC-altered disease as well as a biomarker identified by Virtual Inference of Protein activity by Enriched Regulon analysis (VIPER). For these patients with MYC-altered disease (n = 63), the overall response (OR) rate was 22% with seven responding patients remaining on treatment for approximately two years or longer, and VIPER yielded a three-protein biomarker classification with positive and negative predictive values of ≥85%. Prolonged durations of response were achieved by patients with MYC-altered R/R DLBCL/HGBL treated with single-agent fimepinostat. Combination therapies and/or biomarker-based patient selection strategies may lead to higher response rates in future clinical trials.
法米替尼(CUDC-907)是一种首创的口服小分子组蛋白去乙酰化酶和磷脂酰肌醇 3-激酶抑制剂,在复发/难治性(R/R)弥漫性大 B 细胞淋巴瘤和高级别 B 细胞淋巴瘤(DLBCL/HGBL)患者的 1 期研究中显示出疗效,尤其是那些具有较高 MYC 蛋白表达和/或 MYC 基因重排/拷贝数增加(MYC 改变疾病)的患者。因此,在该患者人群中进行了法米替尼的 2 期研究,共治疗了 66 名符合条件的患者。MYC-免疫组化(IHC)≥40%(n=46)患者的总缓解(OR)率的主要终点为 15%。随后,根据存在 MYC 改变疾病以及通过富集调控分析(VIPER)虚拟推断蛋白活性鉴定的生物标志物,对在 1 期和 2 期研究中接受治疗的患者进行了探索性的汇总分析。对于这些具有 MYC 改变疾病的患者(n=63),OR 率为 22%,有 7 名应答患者持续治疗约两年或更长时间,VIPER 产生了一种具有≥85%阳性和阴性预测值的三蛋白生物标志物分类。接受单药法米替尼治疗的具有 MYC 改变的 R/R DLBCL/HGBL 患者实现了持久的缓解。联合治疗和/或基于生物标志物的患者选择策略可能会在未来的临床试验中带来更高的反应率。