BCR-ABL阴性的MPN的研究进展

Research Progress of BCR-ABL Negative MPN

摘要: 根据2016年WHO修订的骨髓增殖性肿瘤分类标准，经典费城阴性的骨髓增殖性肿瘤(Philadelphia negative myeloproliferative tumor, Ph-MPNs)主要包括真性红细胞增多症(Polycythemia Vera, PV)、原发性血小板增多症(Essential thrombocytopenia, ET)、原发性骨髓纤维化(Primary Myelofibrosis, PMF)。关于Bcr-abl-MPNs的发病机制目前主流观点是JAK-STAT通路激活。由于JAK2V617F、MPL、CALR基因突变的相继发现，二代测序技术的发展，非热点基因应用于三阴性骨髓增殖性肿瘤(Triple negative myeloproliferative tumor, TN-MPN)。使得MPN的治疗进入分子时代，目前能治愈MPN使之达到临床完全缓解的方法只有异基因造血干细胞移植，随着新药的问世以及临床试验阶段，无治疗缓解最终会实现。

Abstract: According to the WHO classification criteria revised in 2016, classical Philadelphia negative myeloproliferative tumor (Ph-MPNs) mainly include Polycythemia Vera (PV), Essential thrombocy-topenia (ET) and Primary Myelofibrosis (PMF). The current mainstream view on the pathogenesis of BCR-ABL-MPNs is JAK-STAT pathway activation. Due to the discovery of JAK2V617F, MPL and CALR mutations and the development of next-generation sequencing technology, non-hotspot genes were applied in Triple negative myeloproliferative tumor (TN-MPN). The treatment of MPN has entered the molecular era. At present, the only way to cure MPN and achieve complete clinical remission is allogeneic hematopoietic stem cell transplantation. With the advent of new drugs and clinical trials, treatment-free remission will eventually be achieved.