经典Phn -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)和原发性骨髓纤维化(PMF)。伴驱动基因n JAK2、n MPL及n CALR突变是MPN的主要诊断标准之一，骨髓组织病理学特征和非驱动基因突变可以辅助进行MPN诊断和患者预后判断。第1代Janus激酶(JAK)2抑制剂芦可替尼，可快速、显著、持续缩小MPN患者脾体积，并缓解机体症状。多项Ⅲ期临床研究结果证实，第2代JAK抑制剂，如fedratinib、momelotinib和pacritinib，在PMF治疗中的疗效良好。除JAK抑制剂外，针对其他靶点的新药，如B细胞淋巴瘤/白血病(BCL)-2/BCL-xL抑制剂navitoclax、端粒酶抑制剂imetelstat和溴结构域和超末端结构蛋白(BET)抑制剂CPI-0610，也在Phn -MPN患者中显示出缩小脾体积，甚至控制骨髓纤维化(MF)程度的疗效。为了提高临床医师对经典Phn - MPN的认识，笔者拟就近年经典Phn -MPN诊断、预后分层、治疗及疗效评价方面的最新研究进展进行阐述。n “,”Classical Phn - myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocytosis (ET) and primary myelofibrosis (PMF). Presence of driver gene mutation of n JAK2, n MPL or n CALR is a major diagnostic criteria of MPN. Histopathological features of bone marrow and mutations in non-driver gene are also of utmost importance for assisting diagnosis and prognosis of patients. The first-generation Janus kinase (JAK)2 inhibitor ruxolitinib has been used in management of patients in terms of controlling splenomegaly and relieving constitutional symptoms rapidly and continuously. Several phase Ⅲ clinical trials have demonstrated good efficacy in patients with PMF treated by the second-generation JAK inhibitors, such as fedratinib, momelotinib and pacritinib. In addition to JAK inhibitors, new agents targeting other targets, such as B-cell lymphoma/ leukemia (BCL) -2 / BCL-xL inhibitor navitoclax, telomerase inhibitor imetelstat and bromodomain and extraterminal domain (BET) inhibitor CPI-0610, also show the therapeutic effects in reduction of spleen volume and improvement in degree of myelofibrosis(MF). To improve understanding of classical Phn - MPN among clinicians, this article will focus on the latest research progresses of diagnosis, risk stratification, treatment and efficacy evaluation for classical Phn - MPN.n