Anticoagulation Therapy Considerations in Factor VII Deficiency.

Eric Paulus, Kathy Komperda, Gabriel Park, Julie Fusco
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Abstract

Factor VII (FVII) deficiency is the most prevalent rare bleeding disorder in the USA and affects approximately 1 out of every 500,000 people. Warfarin inhibits the synthesis of FVII, in addition to other clotting factors. Warfarin is contraindicated in patients with bleeding tendencies or blood dyscrasias; therefore, the literature regarding the use of warfarin in FVII deficiency is very limited. We report a successful re-challenge of warfarin therapy in a patient with FVII deficiency. A 70-year-old woman with FVII deficiency experienced a significant decrease in FVII activity and subsequent vaginal bleeding roughly 5 weeks after starting warfarin for atrial fibrillation. The patient was switched to aspirin therapy. Nearly 4 years later, warfarin therapy was re-attempted by a different haematologist. After 9 months, FVII activity remained in an acceptable range and no bleeding events had occurred. In addition, once the maintenance dose was established, the international normalized ratio remained within the goal range (1.5-2.0) for the majority of assessments. Regarding future considerations, we hypothesize that anticoagulants that do not directly affect FVII, such as the direct oral anticoagulants, would carry less risk of bleeding complications and therefore may be safer alternatives to warfarin to reduce the risk of thromboembolic stroke in patients with atrial fibrillation and FVII deficiency.

因子 VII 缺乏症的抗凝治疗注意事项。
因子 VII(FVII)缺乏症是美国最常见的罕见出血性疾病,每 50 万人中就有大约 1 人患有这种疾病。除其他凝血因子外,华法林还能抑制 FVII 的合成。有出血倾向或血液异常的患者禁用华法林;因此,有关华法林用于 FVII 缺乏症的文献非常有限。我们报告了一名 FVII 缺乏症患者再次挑战华法林治疗的成功案例。一位患有 FVII 缺乏症的 70 岁女性在开始使用华法林治疗心房颤动约 5 周后,FVII 活性显著下降,随后出现阴道出血。患者转用阿司匹林治疗。将近 4 年后,不同的血液科医生再次尝试了华法林疗法。9 个月后,FVII 活性仍在可接受范围内,且未发生出血事件。此外,一旦确定了维持剂量,国际正常化比率在大多数评估中都保持在目标范围(1.5-2.0)内。关于未来的考虑,我们假设不直接影响 FVII 的抗凝剂(如直接口服抗凝剂)发生出血并发症的风险较低,因此可能成为华法林更安全的替代品,以降低心房颤动和 FVII 缺乏患者血栓栓塞性中风的风险。
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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