骨髓增殖性肿瘤伴发卒中的临床分析  

Clinical Analysis of Myeloproliferative Neoplasms with Stroke

作　　者：白雪燕[1] 白贝贝[1] 于小蕊[1] 孙雪飞[1] 朱红[1] 陈烨[1] 

机构地区：[1]首都医科大学附属北京天坛医院血液科,北京100050

出　　处：《中国卒中杂志》2013年第1期31-38,共8页Chinese Journal of Stroke

摘　　要：目的提高对骨髓增殖性肿瘤伴发卒中诊断和治疗的认识。方法收集2008年11月～2011年12月北京天坛医院血液科收治的骨髓增殖性肿瘤伴卒中患者的临床资料、实验室及影像学检查结果 [包括病史、年龄、临床表现、颈部血管彩色多普勒超声、颅脑计算机断层扫描(computed tornography,CT)或磁共振成像(magnetic resonance imaging,MRI)、全血细胞计数、JAK2V617F基因突变检查],并对治疗随访评价。结果骨髓增殖性肿瘤伴卒中16例,其中脑梗死13例,脑出血1例,短暂性脑缺血发作2例;脑梗死患者中4例患者梗死复发。真性红细胞增多症7例,原发性血小板增多症4例,原发性骨髓纤维化5例。7例惠者卒中于骨髓增殖性肿瘤诊断前发生,8例同时诊断,1例骨髓增殖性肿瘤诊断后发生。1 3例有不同程度血细胞升高,13例行。AK2基因突变检测,阳性率76.9%。14例行颈部血管彩色多普勒超声检查均显示颈动脉和锁骨下动脉不同程度增厚、斑块形成伴狭窄。1 6例惠者均服用阿司匹林治疗,其中15例患者同时接受羟基脲和(或)α干扰素治疗。结论本组病例结果提示,卒中惠者需仔细阅读全血细胞计数,重视骨髓增殖性肿瘤的诊断,JAK2基因突变检测有助于骨髓增殖性肿瘤的早期诊断。骨髓增殖性肿瘤患者常伴有颈部血管的结构异常。卒中患者需重视骨髓增殖性肿瘤的诊治。Objective To improve the understanding on the diagnosis and treatment of myeloproliferative neoplasms（MPNs） with stroke. Methods Patients suffering from MPNs with stroke at Department of Hematology of Beijing Tiantan Hospital from November 2008 to December 2011 were included in our study. Their clinical data, laboratory and radiological examination results（including medical history, age, clinical manifestation, color Doppler sonography, brain computed tomography[CT] or magnetic resonance imaging[MRI], complete blood count, and JAK2V617F mutation） were collected. Their treatment and follow-ups were also reviewed. Results There were 16 patients suffering from MPNs with stroke, among whom, 13 with cerebral infarct, 1 with cerebral hemorrhage and 2 with transient ischemic attack（TIA）. Among those with cerebral infarct, 4 underwent relapse. There were 7 cases with polycythemia vera（PV）, 4 with essential thrombocythemia（ET）, and 5 with primary myelofibrosis（PMF）. Seven patients developed stroke before the diagnosis of MPNs. Eight patients were diagnosed stroke and MPNs concurrently. One patient developed stroke after the diagnosis of MPNs. Thirteen patient had blood count increased in different extents. Thirteen patients underwent JAK2 gene mutation detection with a positive rate of 76.9%. Fourteen patients who had received carotid artery color Doppler ultrasound scanning were found the intimae-media thickness of their carotid and subclavian artery increased in different extents, and atherosclerotic plaque with stenosis. All 16 patients were treated with aspirin, and in the meantime 15 managed with hydroxyurea and/or interferon-or. Conclusion The medical records for patients suffering from MPN with stroke suggest that their complete blood count should be carefully reviewed, and more importance should be paid to the diagnosis of MPNs. JAK2V617F mutation detection is helpful to early diagnosis of MPNs. Patients with MPN often develop abnormal structure of neck blood vessel. The diagnosis and treat

关 键 词：骨髓增殖性肿瘤 卒中 血栓形成 JAK2基因突变 治疗 

分 类 号：R733.3[医药卫生—肿瘤]