Increased platelet, leukocyte, and coagulation activation in primary myelofibrosis
Increased platelet, leukocyte, and coagulation activation in primary myelofibrosis
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DOI：
10.1007/s00277-007-0386-3
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发表时间：
2008-04-01
影响因子：
3.5
通讯作者：
Cervantes, Francisco
中科院分区：
文献类型：
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作者：
Alvarez-Larran, Alberto;Arellano-Rodrigo, Eduardo;Cervantes, Francisco
在真性红细胞增多症（PV）和原发性血小板增多症（ET）中已证实血小板和白细胞活化，但在原发性骨髓纤维化（PMF）中这一信息有限。对26例PMF患者的血小板、白细胞、内皮细胞和凝血激活状态进行了评估，并与22名年龄和性别匹配的健康个体的数据进行了比较。研究包括流式细胞术评估血小板p -选择素的表达[在基线和二磷酸腺苷（ADP）、凝血酶和花生四烯酸刺激后]，血小板-中性粒细胞和血小板-单核细胞复合物，以及中性粒细胞和单核细胞中CD11b的表达。此外，通过酶联免疫吸附法测定可溶性p选择素、sCD40L、组织因子、血栓调节素、凝血酶原片段1+2 （F1+2）和d -二聚体。上述参数与患者的临床资料和是否存在JAK2 V617F突变相关。与对照组相比，PMF患者的基线血小板活化水平升高，可溶性p选择素和血小板p选择素的表达水平显著升高，血小板-单核细胞复合物的百分比也较高。中性粒细胞和单核细胞CD11b在JAK2突变患者中的表达明显高于野生型等位基因或对照组。内皮和凝血激活，如血浆血栓调节蛋白和F1+2水平升高所证明的，在PMF中也发现，JAK2突变患者的F1+2值明显高于野生型等位基因患者。综上所述，PMF患者具有与PV和ET相似的血小板、白细胞、内皮细胞和凝血活化。CD11b过表达和F1+2与JAK2突变存在相关。
Platelet and leukocyte activation has been demonstrated in polycythemia vera (PV) and essential thrombocythemia (ET), but such information is limited in primary myelofibrosis (PMF). Platelet, leukocyte, endothelial, and coagulation activation status was assessed in 26 PMF patients and compared with data from 22 age- and sex-matched healthy individuals. Study included flow cytometry assessment of platelet P-selectin expression [at baseline and after adenosine diphosphate (ADP), thrombin and arachidonic acid stimulation], platelet-neutrophil and platelet-monocyte complexes, and CD11b expression in neutrophils and monocytes. Additionally, soluble P-selectin, sCD40L, tissue factor, thrombomodulin, prothrombin fragment 1+2 (F1+2), and D-dimer were measured by enzyme-linked immunosorbent assays. The above parameters were correlated with the patients' clinical data and presence of the JAK2 V617F mutation. Compared with controls, PMF patients had increased baseline platelet activation, as shown by significantly higher levels of soluble and platelet P-selectin expression, and also higher percentages of platelet-monocyte complexes. Neutrophil and monocyte CD11b expression was significantly higher in patients with the JAK2 mutation than in those with wild-type allele or the controls. Endothelial and coagulation activation, as demonstrated by increased plasma levels of thrombomodulin and F1+2, was also found in PMF, with patients with the JAK2 mutation showing significantly higher values of F1+2 than those with wild-type allele. In conclusion, PMF patients have platelet, leukocyte, endothelial, and coagulation activation similar to that in PV and ET. CD11b overexpression and F1+2 are correlated with the presence of the JAK2 mutation.