Department of Hematology, Ege University Medical School, Children's Hospital, Ege Hemophilia Center, Bornova, Izmir, Turkey.
Clin Appl Thromb Hemost. 2013 Jul-Aug;19(4):389-94. doi: 10.1177/1076029612438611. Epub 2012 Mar 5.
Monitoring bypassing agent therapy and observing concordance with clinical hemostasis is crucial in vital hemorrhages and major surgeries in patients with hemophilia having inhibitor.
We aimed to investigate the value of the thrombin generation assay (TGA) and thromboelastography (TEG) for monitoring hemostasis in patients with hemophilia having inhibitor, during supplementation therapy with bypassing agents.
The study group consisted of 7 patients with hemophilia having factor VIII inhibitor. All patients were male. The median age of the participants was 10 years. Age range was 6 to 32 years. The median inhibitor level was 10 Bethesda units (BU), with a range of 5 to 32 BU. A total of 17 bleeding episodes were evaluated. Both TEG and TGA tests were assessed in addition to clinical responses. Assessments were made prior to bypass agent therapy such as recombinant factor VIIa (rFVIIa) or activated prothrombin complex concentrate (aPCC) for bleeding episodes, during the first hour and 24 hours after either intervention in patients.
No relation between clinical response and TGA or TEG parameters was found in patients. There was no difference between clinical responses after rFVIIa and aPCC treatments. However, after aPCC treatment, endogenous thrombin potential and peak thrombin levels and also TEG R, K, and alpha angle degrees were significantly higher.
In conclusion, we found that the clinical effectiveness of bypass therapy in hemophilia cannot be assessed by TGA and TEG.
在患有抑制剂的血友病患者发生危及生命的出血和重大手术时，监测旁路制剂治疗并观察其与临床止血的一致性至关重要。
我们旨在研究在使用旁路制剂进行补充治疗时，血栓生成分析（TGA）和血栓弹力图（TEG）在监测具有抑制剂的血友病患者止血方面的价值。
研究组包括 7 名患有因子 VIII 抑制剂的血友病患者。所有患者均为男性，平均年龄为 10 岁，年龄范围为 6 至 32 岁。参与者的平均抑制剂水平为 10 个 Bethesda 单位（BU），范围为 5 至 32 BU。共评估了 17 次出血事件。除了临床反应外，还评估了 TEG 和 TGA 测试。在出血发作时，在接受旁路制剂治疗（如重组因子 VIIa（rFVIIa）或活化的凝血酶原复合物浓缩物（aPCC））之前以及在患者接受干预后的第 1 小时和 24 小时进行评估。
未发现患者的临床反应与 TGA 或 TEG 参数之间存在关系。rFVIIa 和 aPCC 治疗后的临床反应无差异。然而，在用 aPCC 治疗后，内源性凝血酶潜能和最大凝血酶水平以及 TEG R、K 和 alpha 角度数显著升高。
总之，我们发现旁路治疗在血友病中的临床效果不能通过 TGA 和 TEG 来评估。
