Balancing hemostasis and thrombosis in aging people with hemophilia

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.