[Balancing hemostasis and thrombosis in aging people with hemophilia].

Azusa Nagao
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Abstract

Advances in hemophilia care have markedly improved bleeding control and extended life expectancy, bringing age-related comorbidities to the forefront. Ischemic heart disease (IHD) and other thrombotic events are no longer rare in real-world practice. While observational studies from Europe and North America report that 5% to 8% of patients experience IHD-related events, earlier Japanese retrospective data suggested a much lower burden. In the nationwide prospective ADVANCE Japan cohort of adults with hemophilia aged ≥40 years, IHD-related events have been observed during follow-up, highlighting the need for contemporary evidence from Japan. Moreover, comparisons between predicted and observed events indicate potential miscalibration of general population-derived risk models (e.g., QRISK and the Suita score) when applied to hemophilia. For acute coronary syndromes and PCI, peri-procedural hemostatic management-ensuring adequate factor VIII/IX activity, optimizing access strategy, and coordinating antithrombotic intensity-is essential. Shortened dual antiplatelet therapy regimens supported by trials in high-bleeding-risk populations may be informative, but extrapolation requires individualized assessment. The role of direct oral anticoagulants, including in patients receiving emicizumab, remains uncertain. Multidisciplinary care and shared decision-making are pivotal to balancing bleeding and thrombosis risks in the aging hemophilia population.

[老年血友病患者止血与血栓的平衡]。
血友病护理的进步显著改善了出血控制并延长了预期寿命,使与年龄相关的合并症成为关注的焦点。缺血性心脏病(IHD)和其他血栓事件在现实世界的实践中不再罕见。虽然来自欧洲和北美的观察性研究报告称,5%至8%的患者经历过与ihd相关的事件,但日本早期的回顾性数据表明,负担要低得多。在针对年龄≥40岁的血友病成人的ADVANCE日本全国前瞻性队列研究中,在随访期间观察到与ihd相关的事件,强调需要来自日本的当代证据。此外,预测和观察事件之间的比较表明,在血友病应用时,一般人群衍生的风险模型(例如,QRISK和Suita评分)可能存在校准错误。对于急性冠状动脉综合征和PCI,术中止血管理——确保足够的VIII/IX因子活性、优化通路策略和协调抗血栓强度——是必不可少的。在高危人群中试验支持的缩短双重抗血小板治疗方案可能提供信息,但外推需要个性化评估。直接口服抗凝剂的作用,包括在接受emicizumab治疗的患者中,仍然不确定。多学科护理和共同决策是平衡老年血友病人群出血和血栓风险的关键。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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