發(fā)布時(shí)間：2018-11-07 11:51

【摘要】：目的:探討中國(guó)年輕原發(fā)性血小板增多癥(ET)患者的臨床特點(diǎn)及長(zhǎng)期轉(zhuǎn)歸,建立年輕ET患者特異的血栓預(yù)測(cè)模型,為預(yù)后判斷及治療提供依據(jù)。方法:對(duì)1990年7月1日至2014年12月31日診治的的125例年輕ET患者的病歷資料進(jìn)行回顧性分析。結(jié)果:在全部125例患者中,男37例,女88例,診斷時(shí)中位年齡32(18-40)歲。隨訪中18例(14.4%)出現(xiàn)嚴(yán)重血栓事件。多因素分析表明,JAK2 V617F(HR=8.895,P=0.001)、既往血栓史(HR=8.001,P0.001)及WBC≥12.0×10~9/L(HR=5.225,P=0.002)為血栓事件的獨(dú)立危險(xiǎn)因素。年輕ET患者的血栓發(fā)生率及血栓危險(xiǎn)因素不同于整體ET人群,因此建立了年輕ET特異的血栓預(yù)測(cè)模型,即根據(jù)JAK2V617F(2分)、既往血栓史(2分)和WBC≥12.0×10~9/L(1分),將年輕ET分為低危(0分)、中危(1-2分)和高危(≥3分),3組的無(wú)血栓生存有顯著差異(χ~2=32.223,P0.001)。使用抗血小板聚集藥物可預(yù)防血栓形成(HR=0.081,P0.001),但只有在中、高危患者中,減低血小板藥物可降低血栓發(fā)生率[14.3%(5/35)vs 36.4%(12/33),χ~2=4.416,P=0.036]。7例患者(5.6%)進(jìn)展為骨髓纖維化,其中1例進(jìn)展為骨髓纖維化后又進(jìn)展為急性白血病。僅診斷時(shí)WBC≥15.0×10~9/L是疾病進(jìn)展危險(xiǎn)因素(χ~2=5.434,P=0.020),抗血小板聚集藥物及減低血小板藥物均不能阻止疾病進(jìn)展。結(jié)論:中國(guó)年輕ET患者的血栓發(fā)生率與血栓危險(xiǎn)因素與整體ET患者不同,年輕ET患者特異的血栓預(yù)測(cè)模型對(duì)指導(dǎo)分層治療有重要價(jià)值。
[Abstract]:Objective: to investigate the clinical characteristics and long-term outcome of young patients with (ET) in China, and to establish a specific thrombotic prediction model for young patients with ET, and to provide evidence for prognosis and treatment. Methods: the medical records of 125 young patients with ET from July 1, 1990 to December 31, 2014 were retrospectively analyzed. Results: of the 125 patients, 37 were males and 88 females. The median age of diagnosis was 32 (18-40) years. Severe thrombotic events occurred in 18 cases (14. 4%) during follow-up. Multivariate analysis showed that JAK2 V617F (HR=8.895,P=0.001), past thrombus history (HR=8.001,P0.001) and WBC 鈮，

本文編號(hào)：2316281