Division of Cardiology, Department of Medicine, University of Washington, 1959 NE Pacific St, Box 356422, Seattle, WA, 98195, USA.
Department of Medicine, University of California, San Francisco, San Francisco, CA, USA.
J Thromb Thrombolysis. 2022 Aug;54(2):323-329. doi: 10.1007/s11239-022-02664-y. Epub 2022 May 11.
Therapeutic advances have resulted in increased life expectancy in patients with hemophilia. Consequently, the prevalence of coronary artery disease in this population is increasing. Little is known about the optimal management of acute coronary syndrome in these patients. Current guidelines for the management of this condition are based mainly on expert opinion and generally recommend administration of the clotting factor prior to the anticoagulant, antiplatelet, and interventional therapies. We report a case that illustrates the potential harm that may come from this approach: evolution of non-ST-segment elevation acute coronary syndrome into ST-elevation acute coronary syndrome during the administration of recombinant clotting factor. We review available literature and describe the refined informatics-based guidelines for managing acute coronary syndrome in patients with hemophilia we developed in response to the presented clinical case. We propose adopting this novel informatics-based approach, which aids in the identification and early treatment of these patients, operationalizes timely involvement of hematology experts, and gathers data for further study.
治疗方法的进步使血友病患者的预期寿命延长。因此，该人群中心血管疾病的患病率正在增加。目前对于这些患者急性冠状动脉综合征的最佳治疗方法知之甚少。目前该疾病的管理指南主要基于专家意见，一般建议在抗凝、抗血小板和介入治疗之前使用凝血因子。我们报告了一个病例，说明了这种方法可能带来的潜在危害：在给予重组凝血因子的过程中，非 ST 段抬高型急性冠状动脉综合征进展为 ST 段抬高型急性冠状动脉综合征。我们回顾了现有文献，并描述了为应对所呈现的临床病例而制定的针对血友病患者急性冠状动脉综合征管理的精细化基于信息学的指南。我们建议采用这种新的基于信息学的方法，该方法有助于识别和早期治疗这些患者，及时让血液学专家参与进来，并收集数据进行进一步研究。
