文章：

验证IPSET评分在前纤维化型骨髓纤维化患者血栓形成中的有效性

Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis

原文发布日期：2020-02-25

DOI: 10.1038/s41408-020-0289-2

类型: Article

开放获取: 是

英文摘要：

Pre-fibrotic myelofibrosis (pre-PMF) and essential thrombocythemia (ET) are characterized by similarly increased rate of thrombotic events, but no study specifically analyzed risk factors for thrombosis in pre-PMF. In a multicenter cohort of 382 pre-PMF patients collected in this study, the rate of arterial and venous thrombosis after diagnosis was 1.0 and 0.95% patients/year. Factors significantly associated with arterial thrombosis were age, leukocytosis, generic cardiovascular risk factors, JAK2V617F and high molecular risk mutations, while only history of previous thrombosis, particularly prior venous thrombosis, was predictive of venous events. The risk of total thromboses was accurately predicted by the the international prognostic score for thrombosis in essential thrombocythemia (IPSET) score, originally developed for ET, and corresponded to 0.67, 2.05, and 2.95% patients/year in the low-, intermediate-, and high-risk categories. IPSET was superior to both the conventional 2-tiered score and the revised IPSET in this cohort of pre-PMF patients. We conclude that IPSET score can be conveniently used for thrombosis risk stratification in patients with pre-PMF and might represent the basis for individualized management aimed at reducing the increased risk of major cardiovascular events. Further refinement of the IPSET score in pre-PMF might be pursued by additional, prospective studies evaluating the inclusion of leukocytosis and/or adverse mutational profile as novel variables.
 

摘要翻译： 

骨髓纤维化前期（pre-PMF）与原发性血小板增多症（ET）具有相似的血栓事件发生率，但目前尚无研究专门分析pre-PMF患者血栓形成的风险因素。本研究收集的382例pre-PMF多中心队列数据显示，诊断后动脉与静脉血栓发生率分别为1.0%和0.95%（患者/年）。与动脉血栓显著相关的因素包括年龄、白细胞增多、常规心血管风险因素、JAK2V617F突变及高危分子突变，而仅既往血栓史（特别是静脉血栓史）可预测静脉血栓事件。适用于ET的血栓国际预后评分系统（IPSET）可准确预测pre-PMF患者总体血栓风险，其低危、中危和高危组的发生率分别为0.67%、2.05%和2.95%（患者/年）。在该pre-PMF队列中，IPSET评分优于传统两级评分系统及修订版IPSET。我们得出结论：IPSET评分可便捷用于pre-PMF患者血栓风险分层，并可能作为个体化治疗的基础以降低主要心血管事件风险。未来可通过前瞻性研究进一步优化IPSET评分，评估将白细胞增多和/或不良突变谱作为新增变量的可行性。

原文链接：

Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis