JAK2V617F等位基因负荷与血栓形成：真性红细胞增多症和原发性血小板增多症的直接比较
JAK2V617F allele burden and thrombosis: a direct comparison in essential thrombocythemia and polycythemia vera.
作者信息
Carobbio Alessandra, Finazzi Guido, Antonioli Elisabetta, Guglielmelli Paola, Vannucchi Alessandro M, Dellacasa Chiara M, Salmoiraghi Silvia, Delaini Federica, Rambaldi Alessandro, Barbui Tiziano
机构信息
Hematology Department, Ospedali Riuniti di Bergamo, Bergamo, Italy.
出版信息
Exp Hematol. 2009 Sep;37(9):1016-21. doi: 10.1016/j.exphem.2009.06.006. Epub 2009 Jun 24.
OBJECTIVE
A direct comparison of the incidence and risk factors of major thrombosis in essential thrombocythemia (ET) and polycythemia vera (PV) according to their respective JAK2V617F allele burden is the object of this study.
MATERIALS AND METHODS
We compared the rate (%/patients/year) of major thrombosis in 867 ET patients (57% JAK2V617F) with that of 415 PV patients (all JAK2V617F) and examined risk factors.
RESULTS
Patients with ET wild-type, ET V617F, and PV showed a rate of thrombosis of 1.4%, 2.1%, and 2.7%/patients/year, respectively. The latter was found to progressively increase according to time of diagnosis. Actuarial probability of arterial and venous thrombosis in the first 5 years of diagnosis was roughly similar in the three groups. While in the subsequent periods, the curves of mutated ET patients diverged from wild-type, and after 10 to 15 years the ET-mutated arm approached PV.
CONCLUSION
These findings support the concept of a continuum between ET JAK2 mutated and PV, not only in reference to the hematological phenotype, but also in terms of vascular events.
目的
本研究旨在根据原发性血小板增多症（ET）和真性红细胞增多症（PV）各自的JAK2V617F等位基因负荷，直接比较这两种疾病中主要血栓形成的发生率和危险因素。
材料与方法
我们比较了867例ET患者（57%为JAK2V617F）和415例PV患者（均为JAK2V617F）的主要血栓形成率（%/患者/年），并检查了危险因素。
结果
ET野生型、ET V617F和PV患者的血栓形成率分别为1.4%、2.1%和2.7%/患者/年。发现后者的血栓形成率随诊断时间逐渐增加。三组患者在诊断后的前5年中，动脉和静脉血栓形成的精算概率大致相似。而在随后的时期，ET突变患者的曲线与野生型患者分离，在10至15年后，ET突变组的曲线接近PV组。
结论
这些发现支持ET JAK2突变与PV之间存在连续性的概念，不仅在血液学表型方面，而且在血管事件方面。
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