125例中国年轻原发性血小板增多症患者的临床特点及预后分析

发布时间：2018-11-07 11:51

【摘要】：目的:探讨中国年轻原发性血小板增多症(ET)患者的临床特点及长期转归,建立年轻ET患者特异的血栓预测模型,为预后判断及治疗提供依据。方法:对1990年7月1日至2014年12月31日诊治的的125例年轻ET患者的病历资料进行回顾性分析。结果:在全部125例患者中,男37例,女88例,诊断时中位年龄32(18-40)岁。随访中18例(14.4%)出现严重血栓事件。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)为血栓事件的独立危险因素。年轻ET患者的血栓发生率及血栓危险因素不同于整体ET人群,因此建立了年轻ET特异的血栓预测模型,即根据JAK2V617F(2分)、既往血栓史(2分)和WBC≥12.0×10~9/L(1分),将年轻ET分为低危(0分)、中危(1-2分)和高危(≥3分),3组的无血栓生存有显著差异(χ~2=32.223,P0.001)。使用抗血小板聚集药物可预防血栓形成(HR=0.081,P0.001),但只有在中、高危患者中,减低血小板药物可降低血栓发生率[14.3%(5/35)vs 36.4%(12/33),χ~2=4.416,P=0.036]。7例患者(5.6%)进展为骨髓纤维化,其中1例进展为骨髓纤维化后又进展为急性白血病。仅诊断时WBC≥15.0×10~9/L是疾病进展危险因素(χ~2=5.434,P=0.020),抗血小板聚集药物及减低血小板药物均不能阻止疾病进展。结论:中国年轻ET患者的血栓发生率与血栓危险因素与整体ET患者不同,年轻ET患者特异的血栓预测模型对指导分层治疗有重要价值。
[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，

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