Thrombosis in myeloproliferative disorders: Prevalence, prognostic factors, and the role of leukocytes and JAK2V617F
Thrombosis in myeloproliferative disorders: Prevalence, prognostic factors, and the role of leukocytes and JAK2V617F
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DOI：
10.1055/s-2007-976165
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发表时间：
2007-06-01
影响因子：
5.7
通讯作者：
Elliott, Michelle
中科院分区：
文献类型：
--
作者：
Tefferi, Akyalew;Elliott, Michelle
潜在的骨髓增生性疾病（MPD），特别是真性红细胞增多症（PV）或原发性血小板增多症（ET），是血栓形成的危险因素。考虑到大型选定研究，诊断时，PV和ET的严重血栓形成患病率范围相似，分别为34 - 39%和10 - 29%;随访时，PV和ET的血栓形成相应数字相似，分别为8 - 19%和8 - 31%。在所有情况下，动脉事件比静脉事件更频繁。在PV和ET中，高龄和血栓形成史是复发性血栓形成的独立预测因素。此外，白细胞增多，而不是血小板增多，已被确定为这两种疾病中血栓形成的潜在危险因素。具体的观察结果与实验室证明一致，在这些疾病中，活化的粒细胞和粒细胞-血小板聚集体的数量增加，粒细胞上调血小板P-选择素和组织因子表达，以及羟基脲治疗的抗血栓形成价值。最近，JAK 2功能获得性突变（JAK 2 V617 F）在几乎所有PV患者和50%的ET患者中被描述。目前正在研究这种特定突变或其等位基因负荷的存在是否会改变MPI患者的血栓形成风险。
An underlying myeloproliferative disorder (MPD), especially polycythemia vera (PV) or essential thrombocythemia (ET), is a risk factor for thrombosis. Considering large selected studies, prevalence rates for major thrombosis, at time of diagnosis, range from similar to 34 to 39% for PV and 10 to 29% for ET; the corresponding figures for thrombosis At follow-up are similar to 8 to 19% for PV and 8 to 31% for ET. In all instances, arterial events were more frequent than venous events. In both PV and ET, advanced age and history of thrombosis are independent predictors of recurrent thrombosis. In addition, leukocytosis, but not thrombocytosis, has been identified as a potential risk factor for thrombosis in both diseases. The particular observation is consistent with the laboratory demonstration, in these disorders, of increased number of activated granulocytes and granulocyte-platelet aggregates, upregulation of platelet P-selectin and tissue factor expression by granulocytes, and the antithrombotic value of hydroxyurea therapy. Most recently, a JAK2 gain-of-function mutation (JAK2V617F) was described in virtually all patients with PV and similar to 50% of those with ET. Whether the presence of this specific mutation or its allele burden modifies the risk of thrombosis in patients with MPI)s currently is under investigation.