真性红细胞增多症发病分子机制的研究进展  

Advances of molecular pathogenesis in polycythemia vera

作　　者：王超 周剑峰 陈建春 Wang Chao;Zhou Jianfeng;Chen Jianchun(Tianjin KingMed Center of Clinical Laboratory,Tianjin 300384,Chin)

机构地区：[1]天津金域医学检验所有限公司,300384

出　　处：《国际输血及血液学杂志》2018年第3期267-271,共5页International Journal of Blood Transfusion and Hematology

摘　　要：真性红细胞增多症（PV）是一种病因不明的造血干细胞克隆性疾病。PV患者的常见临床表现为头疼、全身瘙痒、脾大、血栓形成及消化道出血等，PV可进展为骨髓纤维化、急性白血病。PV发病和进展的分子机制与以下多种因素相关，包括多种生长因子及其受体、信号通路及关键酶异常、染色体异常、基因突变和表达异常。PV发病分子机制的揭示有助于为临床寻找新的诊断指标和药物作用靶点，为PV患者的诊断、治疗及预后评价提供参考。笔者拟就这些相关因素在PV中的研究进展进行综述。Polycythemia vera （PV） is a clonal hematopoietic stem cell disorder with unknown etiology. Patients with PV often have headaches, itching, splenomegaly, thrombosis and gastrointestinal bleeding. PV has capability progress to myelofibrosis and acute leukemia. Pathogenic mechanism of PV may be related to many factors, such as growth factors and their receptors, signal pathway and key enzyme abnormalities,chromosome abnormality and gene mutations. The reveal of the molecular mechanism of PV helps to find new diagnostic and medicinal targets in clinic, so as to provide references for diagnosis, treatment and prognosis of PV patients. This article summarizes the progress of these related factors in PV.

关 键 词：红细胞增多症 真性 受体 生长因子 信号传导 染色体畸变 突变 

分 类 号：R555.1[医药卫生—血液循环系统疾病]