Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
Synapse, Maastricht, the Netherlands.
J Thromb Haemost. 2017 May;15(5):868-875. doi: 10.1111/jth.13674. Epub 2017 Apr 13.
Essentials It is unknown if hemophilia patients with atrial fibrillation need anticoagulation. Endogenous thrombin potentials (ETP) in hemophilia patients and patients on coumarins were compared. Severe hemophilia patients had comparable ETP to therapeutic international normalized ratio (INR). In non-severe hemophilia, 33% had higher ETP than therapeutic INR and may need anticoagulation. Click to hear Dr Negrier's perspective on global assays for assessing coagulation SUMMARY: Background It is unknown whether patients with hemophilia A with atrial fibrillation require treatment with vitamin K antagonists (VKAs) to the same extent as the normal population. Objective To compare hemostatic potential in hemophilia patients and patients on VKAs using thrombin generation (TG). Methods In this cross-sectional study, TG, initiated with 1pM tissue factor, was measured in 133 patients with severe (FVIII < 1%, n = 15) and non-severe (FVIII 1-50%, n = 118) hemophilia A, 97 patients on a VKA with an international normalized ratio (INR) ≥ 1.5 and healthy controls. Endogenous thrombin potential (ETP) (nm*min) was compared according to FVIII level (< 1%, 1-19% and 20-50%) with healthy controls and patients with sub-therapeutic INR (1.5-1.9) and therapeutic INR (≥ 2.0). Medians and interquartile ranges (IQRs) were calculated. Results Compared with healthy controls (898 [IQR 803-1004]), both hemophilia patients and patients on VKAs had lower median ETPs at 304 (196-449) and 176 (100-250), respectively. ETP was quite similar in severe hemophilia patients (185 [116-307]) and patients with a therapeutic INR (156 [90-225]). Compared with patients with therapeutic INR, ETP in patients with FVIII 1-19% and patients with FVIII 20-50% was higher at 296 (203-430) and 397 (219-632), respectively. All patients with therapeutic INR had an ETP < 400. Considering this threshold, 93% of severe hemophilia patients, 70% of patients with FVIII 1-19% and 52% of patients with FVIII 20-50% had an ETP < 400. Conclusion In severe hemophilia patients, TG was comparable to that in patients with a therapeutic INR. In one-third of non-severe hemophilia patients, TG was higher. These results suggest that anticoagulation therapy should be considered in a substantial proportion of non-severe hemophilia patients.
目前尚不清楚患有心房颤动的血友病 A 患者是否需要与普通人群一样程度地接受维生素 K 拮抗剂 (VKA) 治疗。目的：使用血栓生成 (TG) 比较血友病患者和 VKA 患者的止血潜能。方法：在这项横断面研究中，用 1pM 组织因子启动 TG 测量，纳入 133 例严重 (FVIII < 1%，n = 15) 和非严重 (FVIII 1-50%，n = 118) 血友病 A 患者、97 例 INR ≥ 1.5 的 VKA 患者和健康对照者。根据 FVIII 水平 (< 1%、1-19%和 20-50%)，将内源性凝血酶潜能 (ETP)(nm*min) 与健康对照者和 INR 低于治疗范围 (1.5-1.9) 和治疗范围 (≥ 2.0) 的患者进行比较。计算中位数和四分位距 (IQR)。结果：与健康对照者 (898[IQR 803-1004]) 相比，血友病患者和 VKA 患者的 ETP 中位数均较低，分别为 304(196-449) 和 176(100-250)。严重血友病患者 (185[116-307]) 和治疗 INR 患者的 ETP 相当。与治疗 INR 患者相比，FVIII 1-19%和 FVIII 20-50%的患者 ETP 分别为 296(203-430) 和 397(219-632)。所有治疗 INR 的患者 ETP 均<400。考虑到这一阈值，93%的严重血友病患者、70%的 FVIII 1-19%患者和 52%的 FVIII 20-50%患者的 ETP<400。结论：在严重血友病患者中，TG 与治疗 INR 患者的 TG 相当。在三分之一的非严重血友病患者中，TG 较高。这些结果表明，抗凝治疗应考虑在相当一部分非严重血友病患者中进行。
