骨髓增殖性肿瘤异质性与克隆演化

Heterogeneity and Clonal Evolution of Myeloproliferative Neoplasms

费城染色体阴性骨髓增殖性肿瘤(myeloproliferative neoplasms,MPNs)是一组以成熟髓系细胞增生为主要特征的慢性血液肿瘤。MPN在遗传、临床表型和肿瘤细胞代谢等多个方面都具有显著的异质性,其克隆演化过程受到造血干细胞突变、骨髓炎症环境和免疫功能失调等多种内、外因素的影响。该文拟从异质性和克隆演化的角度综述MPN发生发展及其调控机制的最新研究进展。

MPNs(myeloproliferative neoplasms)are a group of chronic hematological neoplasms characterized by the proliferation of mature myeloid cells.Patients with MPNs show a significantly greater degree of heterogeneity in genetics,clinical phenotype,and cancer metabolism.The clonal evolutionary process is regulated by various intrinsic and extrinsic factors including mutations in HSCs(hematopoietic stem cells),inflammatory bone marrow microenvironment and immune dysfunction.Here,recent advances in the pathogenesis of MPN from the perspective of heterogeneity and clonal evolution are summarized.