原发性骨髓纤维化发病机制研究进展

Studies on the pathogenesis of primary myelofibrosis

近年来通过对原发性骨髓纤维化（PMF）患者造血细胞基因突变、基因转录后修饰、细胞集落形成以及细胞周期特点等方面的研究，认为PMF是造血干祖细胞恶性克隆性增生性疾病，并将PMF与真性红细胞增多症（PV）、原发性血小板增多症（ET）共同定义为慢性骨髓增生性肿瘤。

In the past decades, genetic lesions, aberrant epigenetic modifications, progenitor colonies formations and cell cycle distribution have been the focus of studies on the pathogenesis of primary myelofibrosis （PMF）. The relative results showed that PMF is a elonal proliferative disease of hematopoietic stem cells. Now it is defined that primary myelofibrosis, polycythemia vera （PV） and essential thrombocythemia （ET） are chronic myeloproliferative neoplasmas（CMPN）.