骨髓增殖性肿瘤疾病进展研究现状和面临的挑战

Research status and challenges of advanced myeloproliferative neoplasms

经典费城染色体(Ph)阴性骨髓增殖性肿瘤(MPN)也称为Ph-MPN,包括真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF)。继发骨髓纤维化(sMF)、继发急性髓系白血病(sAML)是MPN重要的疾病进展。MPN疾病进展后,造血干细胞发生新的克隆演化,导致患者出现耐药,治疗效果欠佳、生存极差。近年来,MPN疾病进展的机制探索及精准诊治受到重视。本文就MPN疾病进展的研究现状进行总结,包括MPN疾病进展的发病机制、危险分层、精准治疗,旨在为探索MPN疾病进展新的诊治方法提供参考。

Classical myeloproliferative neoplasms(MPN),also known as Ph-MPN,includes polycythemia vera(PV),essential thrombocythemia(ET)and primary myelofibrosis(PMF).Secondary myelofibrosis(sMF)and secondary acute myeloid leukemia(sAML)are important disease progressions of MPN.After MPN disease progression,hematopoietic stem cells undergo new clonal evolution,leading to drug resistance,poor treatment effect and poor survival of patients.In recent years,the exploration of the mechanism of disease progression and the precise diagnosis and treatment of MPN have attracted much attention.This article summarizes the research status of MPN disease progression,including the pathogenesis,risk stratification,and precision treatment,in order to provide reference for exploring new diagnosis and treatment methods of MPN disease progression.