原发性血小板增多症的诊断标准及治疗进展

原发性血小板增多症(ET)是骨髓增殖性肿瘤的一种,其特征是血小板水平显著持续性增多而功能异常。近年来认为其发病机制可能与蛋白质酪氨酸激酶2基因V617F突变有关。ET发病较隐匿,进展缓慢。蛋白质酪氨酸激酶2基因V617F突变的发现,传统干扰素、羟基脲减少血小板数目,加上阿司匹林、阿那格雷等药物进行血小板功能干预治疗,均使得ET在诊断及治疗上有了很大突破。

Progress in Diagnostic Criteria and Treatment of Essential Thrombocythaemia

Essential thrombocythaemia(ET)is one of a myeloproliferative neoplasms(MPN)characterized by persistent high level of platelet count and the abnormal function of platelet.It′s pathogenesis may be related to the V617F mutation of JAK-2 gene.In the early time,ET patients may be without any clinical symptoms.Recently,the diagnosis and the treatment of ET have a great advancement because of the discovery of JAK2 V617F mutation,and the connection of the traditional treatment(such as hydroxycarbamide,interferon,et al)to decrease platelet counts and platelet function interventional treatment(such as aspirin,anagrelide,et al).