Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis
Validation of the IPSET score for thrombosis in patients with prefibrotic myelofibrosis
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DOI：
10.1038/s41408-020-0289-2
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发表时间：
2020-02-25
影响因子：
12.8
通讯作者：
Barbui, Tiziano
中科院分区：
文献类型：
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作者：
Guglielmelli, Paola;Carobbio, Alessandra;Barbui, Tiziano
纤维化前骨髓纤维化（pre-PMF）和原发性血小板增多症（ET）的特点是血栓形成事件的发生率相似地增加，但没有研究专门分析PMF前血栓形成的风险因素。在本研究中收集的382例PMF前患者的多中心队列中，诊断后动脉和静脉血栓形成的发生率分别为1.0%和0.95%患者/年。与动脉血栓形成显著相关的因素是年龄、白细胞增多、一般心血管危险因素、JAK 2 V617 F和高分子风险突变，而只有既往血栓形成史，特别是既往静脉血栓形成史，才能预测静脉事件。原发性血小板增多症血栓形成国际预后评分（IPSET）评分（最初用于ET）可准确预测总血栓形成风险，低、中和高风险类别分别对应0.67、2.05和2.95%患者/年。在该队列的PMF前患者中，IPSET上级优于传统的2级评分和修订的IPSET。我们得出结论，IPSET评分可以方便地用于血栓形成危险分层的患者与pre-PMF，并可能代表的基础上，旨在降低主要心血管事件的风险增加的个体化管理。通过额外的前瞻性研究，评估白细胞增多和/或不良突变特征作为新变量的纳入情况，可能会进一步完善PMF前的IPSET评分。
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.