Objective: To evaluate the role of the revised International Prognostic Score of Thrombosis (IPSET-thrombosis) in predicting the occurrence of thrombotic events in Chinese patients with essential thrombocythemia (ET) and to develop a thrombosis predicting model more applicable to Chinese ET patients. Methods: Medical records of 746 adult patients with an initial diagnosis of ET were retrospectively analyzed. Results: The median age at diagnosis was 52 (18-87) years, with 305 males and 441 females. According to the revised IPSET-thrombosis model, the number of very low-, low-, intermediate-, and high-risk patients were 271 (36.3%) , 223 (29.9%) , 63 (8.4%) and 189 (25.3%) , respectively. The four groups exhibited significantly different thrombosis-free survival (χ(2)=72.301, P<0.001) . Thirty-six patients were reclassified as intermediate-risk according to the revised IPSET-thrombosis instead of low-risk as per the original IPSET-thrombosis. Nineteen intermediate-risk patients as per the original IPSET-thrombosis were upgraded to high-risk according to the revised IPSET-thrombosis. Fifty-one high-risk patients as per the original IPSET-thrombosis were reclassified as low-risk in the revised IPSET-thrombosis. It suggests that the revised IPSET-thrombosis potentially avoids over- or under-treatment. In low-risk patients as per the revised IPSET-thrombosis, the rate of thrombosis in patients with cardiovascular risk factors (CVF) was higher than that in those without (16.3% vs 5.2%, χ(2)=5.264, P=0.022) , and comparable with intermediate-risk patients as per the revised IPSET-thrombosis (16.3% vs 14.3%, χ(2)=0.089, P=0.765) . As a result, a new revised IPSET-thrombosis model more applicable to Chinese ET patients was developed in which patients with CVF in the low-risk group as per the revised IPSET-thrombosis were reclassified as intermediate-risk group. Conclusion: For predicting the occurrence of thrombotic events, the revised IPSET-thrombosis model was better than the original IPSET-thrombosis model. The revised IPSET-thrombosis was optimized and a new revised IPSET-thrombosis model more applicable to Chinese ET patients was developed, and the new evidence for risk stratification and treatment of ET in Chinese was provided.
目的：评估修订版国际血栓预测模型（IPSET）在中国原发性血小板增多症（ET）患者中的应用价值，探索适用于中国ET患者的血栓预测模型。 方法：对1982年3月1日至2012年4月30日期间诊治的746例成人ET患者的病历资料进行回顾性分析。 结果：全部746例患者中，男305例，女441例，诊断时中位年龄52（18~87）岁。采用修订版IPSET将患者分组，其中极低危组271例（36.3%）、低危组223例（29.9%）、中危组63例（8.4%）、高危组189例（25.3%），四组的无血栓生存差异有统计学意义（χ(2)=72.301，P<0.001）。修订版IPSET在原版IPSET低危组中区分出36例中危患者，在原版IPSET中危组中区分出19例高危患者，在原版IPSET高危组中区分出51例低危患者，避免了部分患者的治疗不足或过度。修订版IPSET低危组中有心血管危险因素（CVF）者的血栓发生率显著高于无CVF者[16.3%（8/49）对5.2%（9/174），χ(2)=5.264，P=0.022]，而与中危组相当[16.3%（8/49）对14.3%（9/63），χ(2)=0.089，P=0.765]。因此，在修订版IPSET的基础上，低危组中伴CVF者被划分至中危组，即为适于中国ET患者的修订版IPSET，此模型能区分出更多的血栓患者。 结论：修订版IPSET在血栓预测方面优于原版IPSET，将修订版IPSET优化后得到了更适于中国ET患者的修订版IPSET，为中国ET患者的分层治疗提供了依据。.
Keywords: Mutation; Risk stratification; Thrombocythemia, Essential; Thrombosis.