Haematopoietic Stem Cell Biology Laboratory, Medical Research Council Molecular Haematology Unit, Medical Research Council Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.
NIHR Biomedical Research Centre, University of Oxford, Oxford, UK.
Nat Genet. 2023 Sep;55(9):1531-1541. doi: 10.1038/s41588-023-01480-1. Epub 2023 Sep 4.
Understanding the genetic and nongenetic determinants of tumor protein 53 (TP53)-mutation-driven clonal evolution and subsequent transformation is a crucial step toward the design of rational therapeutic strategies. Here we carry out allelic resolution single-cell multi-omic analysis of hematopoietic stem/progenitor cells (HSPCs) from patients with a myeloproliferative neoplasm who transform to TP53-mutant secondary acute myeloid leukemia (sAML). All patients showed dominant TP53 'multihit' HSPC clones at transformation, with a leukemia stem cell transcriptional signature strongly predictive of adverse outcomes in independent cohorts, across both TP53-mutant and wild-type (WT) AML. Through analysis of serial samples, antecedent TP53-heterozygous clones and in vivo perturbations, we demonstrate a hitherto unrecognized effect of chronic inflammation, which suppressed TP53 WT HSPCs while enhancing the fitness advantage of TP53-mutant cells and promoted genetic evolution. Our findings will facilitate the development of risk-stratification, early detection and treatment strategies for TP53-mutant leukemia, and are of broad relevance to other cancer types.
了解肿瘤蛋白 53(TP53)突变驱动的克隆进化和随后转化的遗传和非遗传决定因素,是设计合理治疗策略的关键步骤。在这里,我们对转化为 TP53 突变型继发性急性髓系白血病(sAML)的骨髓增生性肿瘤患者的造血干细胞/祖细胞(HSPC)进行等位基因解析单细胞多组学分析。所有患者在转化时均表现出显性 TP53“多击”HSPC 克隆,白血病干细胞转录特征在独立队列中对 TP53 突变型和野生型(WT)AML 的不良预后具有很强的预测性。通过对连续样本、先存 TP53 杂合克隆和体内扰动的分析,我们证明了慢性炎症的一种迄今未被认识的作用,它抑制了 TP53 WT HSPCs,同时增强了 TP53 突变细胞的适应性优势,并促进了遗传进化。我们的研究结果将有助于为 TP53 突变型白血病制定风险分层、早期检测和治疗策略,并与其他癌症类型具有广泛的相关性。