JAK2V617F突变性原发性血小板增多症并缺血性卒中10例临床特征分析  

Clinical characteristics analysis of 10 paitents with JAK2 V617F-positive essential thrombocythemia complicated with ischaemic stroke

作　　者：詹自雄[1] 陈宏[1] 蓝梅平 ZHAN Zixiong;CHEN Hong;LAN Meiping(Department of Neurology,Fujian Provincial Hospital,Fujian Medical University Affiliated Provincial Teaching Hospital,Fuzhou,Fujian,350001,China;Department of Neurology,Wuping County Hospital,Longyan,Fujian,364300,China)

机构地区：[1]福建省立医院神经内科/福建医科大学省立临床医学院,福建福州350001 [2]武平县医院神经内科,福建龙岩364300

出　　处：《当代医学》2024年第8期157-161,共5页Contemporary Medicine

基　　金：福建省卫生计生委青年科研课题基金(2015/1/8)。

摘　　要：目的分析JAK2V617F突变性原发性血小板增多症(ET)并缺血性卒中的临床特征。方法回顾性分析2013年7月至2021年5月福建省立医院收治的7例及武平县医院收治的3例JAK2V617F突变性ET合并缺血性卒中患者的临床资料,分析患者临床特征(临床表现、实验室检测、骨髓细胞学)、影像学及治疗转归情况。结果10例JAK2V617F突变性ET合并缺血性卒中患者中,多发性脑梗死7例,单发性脑梗死2例,短暂性脑缺血发作1例。MRI显示3例患者病灶存在出血转化。9例脑梗死患者行血管评估,7例存在大动脉中重度狭窄或者闭塞。1例超急性期就诊者给予阿替普酶静脉溶栓后症状好转。二级预防:6例使用抗血小板、联合骨髓增生抑制剂,病情改善;3例起初仅用抗血小板治疗,病情反复,加用羟基脲治疗后2例病情改善。结论JAK2V617F突变性ET常引起大动脉粥样硬化狭窄性、多发性脑梗死且病灶较易出血,在抗血小板的基础上加用骨髓增生抑制剂可能有利于预防病情复发。Objective To analyze the clinical characteristics of JAK2 V617F-positive essential thrombocythemia(ET)complicated with isch-aemic stroke.Methods The clinical data of 7 patients treated in Fujian Provincial Hospital and 3 patients treated in Wuping County Hospital from July 2013 to May 2021 whose JAK2 V617F-positive ET complicated with ischemic stroke were retrospectively analyzed,The clinical characteristics(clinical manifestations,laboratory tests,bone marrow cytology),imaging,treatment and outcome of the patients were analyzed.Results Among 10 patients with JAK2V617F-positive ET complicated with ischemic stroke,7 cases had multiple cerebral infarction,2 cases had single cerebral infarc-tion and 1 cases had transient ischemic attack.MRI showed hemorrhagic transformation in the lesion in 3 patients.Vascular evaluation was per-formed in 9 patients with cerebral infarction,and 7 patients had moderate to severe stenosis or occlusion of large arteries.The symptoms of 1 patient in hyperacute stage improved after intravenous thrombolysis with alteplase.Secondary prevention,6 cases were treated with antiplatelet and com-bined with myeloproliferative inhibitor,and the condition was improved;3 cases were treated with antiplatelet therapy at first,but the condition was repeated,2 cases were improved after adding hydroxyurea treatment.Conclusion JAK2 V617F-positive ET often causes atherosclerotic stenosis,multiple cerebral infarction and easy bleeding in the lesion,the addition of antiplatelet and myeloproliferative inhibitors may be beneficial to prevent the recurrence of the disease.

关 键 词：原发性血小板增多症 JAK2V617F突变 卒中 影像学 

分 类 号：R743.3[医药卫生—神经病学与精神病学]