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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,P<0.001)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,P<0.001)。使用抗血小板聚集药物可预防血栓形成(HR=0.081,P<0.001),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。
Objective: To investigate the clinical features and long-term prognosis of patients with young primary thrombocystosis (ET) in China and to establish a thrombosis-specific model of thrombosis in young patients with ET, providing evidence for prognosis and treatment. Methods: The clinical data of 125 young ET patients diagnosed and treated from July 1, 1990 to December 31, 2014 were retrospectively analyzed. Results: Of the 125 patients, 37 were males and 88 were females with a median age of 32 (18-40) years at diagnosis. During follow-up, 18 patients (14.4%) had severe thrombotic events. Multivariate analysis showed that the thrombus of JAK2 V617F (HR = 8.895, P = 0.001), previous thrombus history (HR = 8.001, P <0.001) and WBC≥12.0 × 10 ~ 9 / L Independent risk factors for the incident. Thrombosis and thrombotic risk factors in young patients with ET are different from those in the general ET population. Therefore, a thrombotic prediction model for young ET was established based on JAK2V617F (2 points), previous thrombus history (2 points) and WBC≥12.0 × 10 ~ There were significant differences in thrombosis-free survival between the three groups (χ ~ 2 = 9 / L, 1 point) 32.223, P <0.001). The use of antiplatelet drugs prevented thrombosis (HR = 0.081, P <0.001). However, platelet drug therapy reduced the incidence of thrombosis only in mid- and high-risk patients [14.3% (5/35) vs 36.4% 33), χ ~ 2 = 4.416, P = 0.036]. Seven patients (5.6%) progressed to myelofibrosis, one of which progressed to myeloid leukemia with progression to myelofibrosis. Only WBC≥15.0 × 10 ~ 9 / L at diagnosis was the risk factor for disease progression (χ ~ 2 = 5.434, P = 0.020). Both antiplatelet drugs and platelet lowering drugs could not prevent the disease progression. CONCLUSION: Thrombosis and thrombotic risk factors in young Chinese patients with ET are different from those in overall ET. The specific thrombosis prediction model in young patients with ET is of great value in guiding the stratification treatment.