炎症与骨髓增殖性肿瘤的研究现状

Research status on inflammation and myeloproliferative neoplasm

骨髓增殖性肿瘤(MPN)是一组以骨髓一系或多系髓细胞增殖为特征的克隆性造血干细胞疾病,其中经典型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),即Ph-MPN。MPN驱动基因Janus激酶(JAK)2、促血小板生成素受体(MPL)和钙网蛋白(CALR)基因突变,均可激活JAK/信号转导和转录激活因子(STAT)信号通路,上调MPN患者的炎症因子表达水平,形成炎症微环境,并促进克隆细胞不断增殖,导致患者体质症状加重、血栓形成、易向骨髓纤维化(MF)和急性白血病(AL)进展,预后不良。近年,炎症在MPN中的作用逐渐受到重视。笔者拟就炎症对MPN发生和演变、症状负荷、预后及治疗影响的研究现状进行总结,旨在为探索MPN患者新型预后分层标志物和治疗靶点提供参考。

Myeloproliferative neoplasm(MPN)is clonal hematopoietic stem cell disorder characterized by abnormal proliferation of one or more myeloid lineages.Classic MPN include polycythemia vera(PV),essential thrombocythemia(ET)and primary myelofibrosis(PMF),also known as Ph-MPN.Janus kinase(JAK)2,thrombopoietin receptor(MPL)and calreticulin(CALR)gene mutation can activate JAK/signal transducer and activator of transcription(STAT)signaling pathways,and significantly upregulate expression level of inflammatory factors.The formation of an inflammatory microenvironment is conducive to continuous proliferation of malignant clones,which lead to aggravation of constitutional symptoms,thrombosis,high risk progression to myelofibrosis(MF)and acute leukemia(AL),and poor prognosis of patients with MPN.In recent years,the role of inflammation in MPN has gradually attracted attention.This article will summarize research status between inflammation and MPN from several aspects that involve occurrence and evolution,symptom load,prognosis and treatment,in order to provide new prognostic stratification markers and therapeutic targets for patients with MPN.