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首页> 外文期刊>Blood: The Journal of the American Society of Hematology >JAK2V617F mutation and hydroxyurea treatment as determinants of immature platelet parameters in essential thrombocythemia and polycythemia vera patients.
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JAK2V617F mutation and hydroxyurea treatment as determinants of immature platelet parameters in essential thrombocythemia and polycythemia vera patients.

机译:在原发性血小板增多症和真性红细胞增多症患者中,JAK2V617F突变和羟基脲治疗可作为未成熟血小板参数的决定因素。

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摘要

Immature platelets (IPFs), which are hemostatically more active than mature platelets, have been found elevated in essential thrombocythemia and polycythemia vera, 2 myeloproliferative neoplasms (MPN) characterized by an increased risk of thrombosis. It is not known whether the IPF levels are influenced by pathogenetic factors, including JAK2V617F mutational status, or by treatment regimen. To address this point, in 46 essential thrombocythemia and 38 polycythemia vera consecutive patients, we measured IPF and correlated the results to JAK2V617F mutation and myelosuppressive treatment with hydroxyurea. This analysis provides 2 new elements regarding IPF and MPN. The first finding is that the JAK2V617F mutation is linked to the quantity of IPF in patients with MPN, which might contribute to the prothrombotic phenotype in these patients. The second finding is that IPF is susceptible to myelosuppressive treatment, which may additionally explain the favorable effect of hydroxyurea therapy on MPN outcome as well as the associated thrombotic risk.
机译:已发现,在成熟的血小板增多症和真性红细胞增多症中2种骨髓增生性肿瘤(MPN)的血栓形成风险增高,未成熟的血小板(IPFs)具有比成熟血小板更高的止血活性。尚不清楚IPF水平是否受致病因素(包括JAK2V617F突变状态)或治疗方案的影响。为了解决这一点,在46例原发性血小板增多症和38例连续性真性红细胞增多症患者中,我们测量了IPF并将结果与​​JAK2V617F突变和羟基脲的骨髓抑制治疗相关联。该分析提供了两个有关IPF和MPN的新元素。第一个发现是,JAK2V617F突变与MPN患者的IPF数量有关,这可能有助于这些患者的血栓前表型。第二个发现是IPF对骨髓抑制治疗敏感,这可能另外解释了羟基脲治疗对MPN结局以及相关血栓形成风险的有利作用。

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