Division of Immunohematology and Transfusion Medicine, Ospedali Riuniti di Bergamo, Bergamo, Italy.
Blood. 2011 Sep 1;118(9):2599-601. doi: 10.1182/blood-2011-02-339655. Epub 2011 Jul 12.
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.
不成熟血小板（IPF）比成熟血小板更具有止血活性，在特发性血小板增多症和真性红细胞增多症中升高，这两种骨髓增殖性肿瘤（MPN）以血栓形成风险增加为特征。目前尚不清楚 IPF 水平是否受包括 JAK2V617F 突变状态在内的发病因素或治疗方案的影响。为了解决这一问题，我们在 46 例特发性血小板增多症和 38 例真性红细胞增多症连续患者中测量了 IPF，并将结果与 JAK2V617F 突变和羟基脲的骨髓抑制治疗相关联。该分析提供了关于 IPF 和 MPN 的 2 个新元素。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 易受骨髓抑制治疗的影响，这可能进一步解释了羟基脲治疗对 MPN 结果以及相关血栓风险的有利影响。
