Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Kingston, ON, Canada.
Haemophilia. 2009 Sep;15(5):1126-34. doi: 10.1111/j.1365-2516.2009.02037.x. Epub 2009 Jun 1.
The heterogeneity among severe haemophilia A patients reflects on variable tendencies for bleeding and also variable responses to FVIII therapy. This variability cannot be detected or predicted by routine coagulation tests. Thromboelastography (TEG) has recently been evaluated for assessing hemostatic patterns in haemophiliacs and proved valuable in monitoring therapy and/or prophylaxis, however, usually only in limited small case series. Exercise is an important component of overall haemophilia care, however, in severe haemophiliacs there is an increased risk of bleeding. The availability of a validated hemostatic test to evaluate the influence of exercise would be advantageous. This study has used TEG analysis to evaluate the global hemostatic status of a group of severe haemophilia A dogs at rest and after a standardized period of exercise. The study demonstrated significant inter and intra-individual variations based on TEG patterns at rest and following acute exercise as well as significant improvement of global hemostasis after exercise in the majority of tested dogs. The study supports the utilization of TEG in assessment of the hemostatic pattern in severe haemophilia A and provides a potential for utilizing TEG evaluation in managing exercise regimens for haemophilia care.
重度 A 型血友病患者之间存在异质性,表现在出血倾向的差异和对 FVIII 治疗的反应差异。这些变异性不能通过常规凝血测试检测或预测。血栓弹力描记术(TEG)最近已被评估用于评估血友病患者的止血模式,并已被证明在监测治疗和/或预防方面具有价值,但通常仅在有限的小案例系列中进行。运动是整体血友病治疗的重要组成部分,然而,在重度血友病患者中,出血的风险增加。如果有一种经过验证的止血测试来评估运动的影响,那将是有利的。本研究使用 TEG 分析评估了一组重度 A 型血友病犬在休息和经过标准化运动后的整体止血状态。研究表明,根据 TEG 模式,休息时和急性运动后存在显著的个体间和个体内差异,以及大多数受试犬在运动后整体止血功能显著改善。该研究支持在评估重度 A 型血友病的止血模式中使用 TEG,并为在血友病管理中利用 TEG 评估来管理运动方案提供了潜力。