摘要
真性红细胞增多症（PV）是一种累及造血干细胞的慢性克隆性骨髓增殖性疾病,主要临床表现为疲劳、瘙痒及脾大等,易并发血栓栓塞,并有高风险向骨髓纤维化及急性白血病转化.自2005年发现JAK2 V617F突变以来,PV的发病机制、诊断及治疗研究取得了很大进展,表观遗传学标记的改变在PV疾病进展中扮演着重要角色,针对表观遗传学改变的药物有望提高PV患者的生命质量.
Polycythemia vera （PV） is a chronic clonal myeloproliferative disorder of hematopoietic stem cell characterized by a pronounced symptom burden, including fatigue, pruritus, and symptomatic splenomegaly, along with an increased risk of thrombosis and the potential for evolution to myelofibrosis （MF） and secondary acute myeloid leukemia. Research on pathogenesis, diagnosis and treatment of PV has made a great progress since the discovery of JAK2 V617F mutation in 2005. The change of epigenetics plays an important role in the progression of PV, and the drugs for apparent genetic alteration may improve the life quality of PV patients.
作者
李亚茹
陈伟
徐开林
Li Yaru Chen Wei Xu Kailin(Department of Pathology, the Affiliated Hospital of Xuzhou Medical University, Xuzhou 221002, China)
出处
《白血病．淋巴瘤》
CAS
2017年第7期440-444,共5页
Journal of Leukemia & Lymphoma
基金
国家自然科学基金（81300441、81270637）
中国博士后基金面上项目（2016M590507）
江苏省自然科学基金面上项目（BK20161177）
