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经典的BCR/ABL阴性的骨髓增殖性肿瘤(MPN),包括真性红细胞增多症(PV)、原发性血小板增多症(ET)、原发性骨髓纤维化(PMF),是一组起源于多能造血干细胞的恶性骨髓增殖性疾病,且都存在Janus激酶(JAK)2 V617F、JAK2基因第12号外显子、钙网蛋白(CALR)、血小板生成素受体MPL W515L/K等基因突变,这些遗传学改变与MPN发病机制的关系尚未完全明确。2008年,世界卫生组织(WHO)重新修订了MPN的诊断标准,将MPD更名为骨髓增殖性肿瘤(MPN),JAK2等基因突变也被收入其中。MPN既往药物治疗主要包括,羟基脲和/或a干扰素及沙利度胺,而近年来有一些新药尤其是靶向药物的疗效颇具前景,已有部分药物应用于MPN的临床治疗,如日前刚于我国上市的JAK2抑制剂芦可替尼,为MPN的治疗提出了一种新的治疗选择。本文将着重介绍MPN的发病机制及治疗的最新研究进展。
Classical BCR / ABL-negative myeloproliferative tumors (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) Can hematopoietic stem cell malignant myeloproliferative diseases, and there are Janus kinase (JAK) 2 V617F, JAK2 exon 12, calreticulin (CALR), thrombopoietin receptor MPL W515L / K and other genetic mutations, these The relationship between genetic changes and the pathogenesis of MPN has not yet been fully understood. In 2008, the World Health Organization (WHO) revised the diagnostic criteria of MPN, changed the name of MPD to myeloproliferative neoplasm (MPN), and gene mutations such as JAK2 were also included. MPN previous drug therapy mainly includes hydroxyurea and / or alpha interferon and thalidomide. In recent years, some new drugs, especially targeted drugs, have shown promising results. Some of the drugs have been used in the clinical treatment of MPN, Just listed in China, Lakitinib JAK2 inhibitor, for the treatment of MPN proposed a new treatment options. This article will focus on the pathogenesis of MPN and treatment of the latest research progress.