Myeloproliferative neoplasms (MPNs) are hematological diseases that are driven by somatic mutations in hematopoietic stem and progenitor cells. These mutations include JAK2, CALR and MPL mutations as the main disease drivers, mutations driving clonal expansion, and mutations that contribute to progression of chronic MPNs to myelodysplasia and acute leukemia. JAK-STAT pathway has played a central role in the disease pathogenesis of MPNs. Mutant JAK2, CALR or MPL constitutively activates JAK-STAT pathway independent of the cytokine regulation. Symptomatic management is the primary goal of MPN therapy in ET and low-risk PV patients. JAK2 inhibitors and interferon-α are the established therapies in MF and high-risk PV patients.

中文翻译：

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阐明骨髓增生性肿瘤的分子病理生理学及其在治疗决策中的应用方面的进展。

骨髓增生性肿瘤（MPN）是由造血干细胞和祖细胞的体细胞突变驱动的血液系统疾病。这些突变包括作为主要疾病驱动因素的JAK2，CALR和MPL突变，驱动克隆扩增的突变，以及导致慢性MPN演变为骨髓增生异常和急性白血病的突变。JAK-STAT途径在MPN的发病机理中起着核心作用。突变的JAK2，CALR或MPL组成性地激活JAK-STAT途径，而与细胞因子的调节无关。对症治疗是ET和低危PV患者MPN治疗的主要目标。JAK2抑制剂和干扰素-α是MF和高危PV患者的公认疗法。