真性红细胞增多症的临床特征及血栓栓塞并发症危险因素分析  

Analysis of clinical characteristics of polycythemia vera and the risk factors for thromboembolism

作　　者：李亚茹 李德鹏 徐开林 LI Yaru;LI Depeng;XU Kailin(The Affiliated Hospital of Xuzhou Medical University, Xuzhou, 221000, China)

机构地区：[1]徐州医科大学附属医院,徐州221000

出　　处：《临床血液学杂志》2018年第3期353-358,共6页Journal of Clinical Hematology

基　　金：江苏省卫生计生委科研课题(No:H201629)

摘　　要：目的:总结真性红细胞增多症(PV)患者的临床特点、实验室检查结果,分析PV患者血栓栓塞并发症的危险因素,为筛选高危人群、防治并发症提供依据。方法:选取确诊的107例PV患者的临床资料进行回顾性分析总结。结果:(1)JAK2/V617F突变阳性的PV患者,年龄、血小板计数明显高于阴性者(P<0.05)。(2)107例患者中,PT延长41例(38.32%),APTT延长70例(65.42%),TT延长40例(37.38%),PTA降低74例(69.16%),INR升高38例(35.51%),FIB降低27例(25.23%)。(3)共88例患者检测了乳酸脱氢酶(LDH),均值为355.66U/L,明显大于正常高限(240U/L),共89例患者检测了空腹血糖,均值为4.44mmol/L,其中30例(33.71%)空腹血糖低于正常低限(3.8mmol/L),12例可诊断为低血糖症(空腹血糖<2.8mmol/L)。(4)Logistic多因素分析显示,高血小板水平、无脾肿大、JAK2/V617F基因突变阳性是PV患者发生血栓栓塞的独立危险因素(P<0.05)。采用Kaplan-Meier方法计算患者5年无血栓生存率显示,有脾肿大与无脾肿大患者5年无血栓生存率分别为(81.20±0.56)%、(46.30±0.85)%(P<0.05)。JAK2/V617F突变阴性与阳性患者5年无血栓生存率分别为(84.20±0.72)%、(56.30±1.23)%(P<0.05)。结论:JAK2/V617F基因突变阳性的PV患者,年龄及血小板计数明显高于阴性者,且更易并发血栓性疾病。PV患者多合并凝血功能异常,包括PT、APTT延长,FIB降低等。大部分PV患者血清LDH水平增高,部分患者合并未察觉低血糖症。JAK2/V617F基因突变阳性、血小板计数>450×109/L、无脾肿大的患者,更易并发血栓性疾病。Objective：To summarize clinical features and laboratory test results of patients with polycythemia vera（PV）,and analyze risk factors of thromboembolic complications in PV patients,in order to provide basis for screening high-risk groups and prevention of complications.Method：Clinical data of 107 patients diagnosed with PV were retrospectively analyzed.Result：（1）According to whether the existence of JAK2/V617 Fmutation,PV patients could be divided into two groups,the age,platelet count of positive group was obviously higher than those of negative group（P〈0.05）;（2）Of all the 107 patients,PT prolonged in 41 cases（38.32%）,APTT prolonged in 70 cases（65.42%）,TT prolonged in 40 cases（37.38%）,the PTA decreased in 74 cases（69.16%）,INR increased in38 cases（35.51%）,FIB decreased in 27 cases（25.23%）;（3）A total of 88 cases received the test of LDH,averaged355.66 U/L,it was significantly higher than the normal range（240 U/L）.Eighty-nine cases tested the fasting glucose,averaged 4.44 mmol/L,and 30 cases belowed the normal lower limit（3.8 mmol/L,33.71%）,12 cases diagnosed as hypoglycemia（fasting glucose 〈02.8 mmol/L）.（4）The Logistic multi-factors analysis showed the high level of platelet,nonsplenomegaly and JAK2/V617 Fgene mutation positive were the independent risk factors for the development of PV patients occurred thromboembolism（P〈0.05）.Analyses of the thrombosis-free survival were conducted by Kaplan-Meier method,it showed the 5-year thrombosis-free survival of patients with and without splenomegaly were（81.20＋0.56）% and（46.30＋0.85）% respectively（P〈0.05）.The 5-year thrombosisfree survival of patients with and without JAK2/V617 F mutation were（84.20＋0.72）% and（56.30＋1.23）%respectively（P〈0.05）.Conclusion：The PV patients with JAK2/V617 Fgene mutation are significantly higher in age and platelet count than the negative patients,and are more prone to have thrombotic diseases.Patients with PV are more complicated wit

关 键 词：真性红细胞增多症 JAK2/V617F 血栓栓塞 

分 类 号：R555.1[医药卫生—血液循环系统疾病]