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首页> 外文期刊>British Journal of Haematology >Clot lysis phenotype and response to recombinant factor VIIa in plasma of haemophilia A inhibitor patients
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Clot lysis phenotype and response to recombinant factor VIIa in plasma of haemophilia A inhibitor patients

机译：A型血友病抑制剂患者血浆的凝血裂解表型和对重组因子VIIa的反应

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摘要

Recombinant activated factor VII (rFVIIa) is a haemostatic agent that is used for the treatment of haemophilia A patients with inhibitors. However, clinical response to rFVIIa is variable and unpredictable with currently available assays. We investigated the anti-fibrinolytic effects of rFVIIa in relation to thrombin generation (TG) and other haemostatic parameters in haemophilia A patients with inhibitors. After addition of rFVIIa to plasma, the clot-lysis assay, TF-dependent TG, TF-independent TG and parameters involved in coagulation, anticoagulation and fibrinolysis were assessed. The clot-lysis test distinguished two groups of patients: a group with a normal and a group with impaired anti-fibrinolytic response to rFVIIa. Our results showed a dose-dependent increase in TF-dependent TG and TF-independent TG in all individuals. There was a significant difference in TF-independent TG parameters between the normal and impaired response groups. In addition, there was a difference between the normal and impaired response group in prothrombin time, which could be explained by significantly higher levels of coagulation factors in the normal response group, and soluble thrombomodulin. In conclusion, we observed different in vitro responses following rFVIIa addition in plasma of patients with haemophilia A and inhibitors, which could be partially attributed to levels of procoagulant proteins and soluble thrombomodulin.
机译：重组活化因子VII（rFVIIa）是止血剂，用于治疗具有抑制剂的A型血友病患者。但是，对rFVIIa的临床反应是可变的，并且用当前可用的测定方法无法预测。我们研究了rFVIIa与凝血酶生成（TG）和其他具有抑制剂的血友病A患者的其他止血参数有关的抗纤维蛋白溶解作用。将rFVIIa添加至血浆后，评估凝块溶解测定，TF依赖性TG，TF依赖性TG以及涉及凝血，抗凝和纤溶的参数。凝块溶解试验区分出两组患者：一组正常，另一组对rFVIIa的抗纤维蛋白溶解反应受损。我们的结果显示，所有个体中TF依赖性TG和TF依赖性TG的剂量依赖性增加。在正常和受损反应组之间，与TF无关的TG参数存在显着差异。另外，正常组和受损反应组之间的凝血酶原时间有所不同，这可以通过正常响应组中凝血因子和可溶性血栓调节蛋白的水平明显升高来解释。总而言之，我们观察到患有A型血友病和抑制剂的患者血浆中添加rFVIIa后的体外反应不同，这可能部分归因于促凝蛋白和可溶性血栓调节蛋白的水平。

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