Hematology Department, Hospital Universitario Virgen del Rocio, Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Hematology Department, Hospital Regional Universitario de Málaga, Málaga, Spain.
Eur J Haematol. 2022 Dec;109(6):686-695. doi: 10.1111/ejh.13853. Epub 2022 Sep 12.
Activated prothrombin complex concentrate (aPCC) is a bypassing agent indicated to treat bleeds in patients with acquired hemophilia A (AHA). Nevertheless, its efficacy and safety in the real-world setting have not often been addressed.
We report the experience of Spanish reference centers for coagulation disorders and from acquired hemophilia Spanish Registry (AHASR) from August 2012 to February 2021. Follow-up period of 30 days after aPCC withdrawal.
Thirty patients with a median age of 70 years old, suffering from 51 bleeds treated with aPCC were finally evaluated. As first-line treatment, aPCC stopped bleeding in 13 of 14 (92.9%) cases. aPCC as the second line after recombinant factor VIIa failure, stopped bleeding in all cases. In 17 patients, aPCC was used far from initial bleed control as prophylaxis of rebleeding with 94% effectiveness. No thromboembolic episodes were communicated. One patient developed hypofibrinogenemia, which did not prevent aPCC from halting bleeding. No other serious adverse events possibly or probably associated with aPCC were reported.
This data support aPCC as hemostatic treatment in AHA with high effectiveness and excellent safety profile in acute bleeds and as extended use to prevent rebleedings, even in aging people with high cardiovascular risk.
活化的凝血酶原复合物浓缩物（aPCC）是一种旁路制剂，用于治疗获得性血友病 A（AHA）患者的出血。然而，其在真实环境中的疗效和安全性尚未得到广泛关注。
我们报告了西班牙凝血障碍参考中心和获得性血友病西班牙登记处（AHASR）自 2012 年 8 月至 2021 年 2 月的经验。在停用 aPCC 后进行 30 天的随访。
最终评估了 30 名年龄中位数为 70 岁的患者，他们患有 51 例接受 aPCC 治疗的出血。作为一线治疗，aPCC 成功止血 14 例中的 13 例（92.9%）。在 rFVIIa 治疗失败后作为二线治疗，aPCC 成功止血所有病例。在 17 名患者中，aPCC 远未达到初始出血控制，作为预防再出血的预防性治疗，有效率为 94%。未报告血栓栓塞事件。1 名患者出现低纤维蛋白原血症，但这并未阻止 aPCC 止血。未报告其他可能或可能与 aPCC 相关的严重不良事件。
这些数据支持 aPCC 作为 AHA 急性出血的止血治疗，具有高效性和出色的安全性，并且可作为延长使用的药物预防再出血，即使在伴有高心血管风险的老年人群中也是如此。
