HemaSphere (Jun 2023)

Antithrombotic Treatment in Patients With Hemophilia: an EHA-ISTH-EAHAD-ESO Clinical Practice Guidance

  • Roger E.G. Schutgens,
  • Victor Jimenez-Yuste,
  • Miguel Escobar,
  • Anna Falanga,
  • Bruna Gigante,
  • Robert Klamroth,
  • Riitta Lassila,
  • Frank W.G. Leebeek,
  • Michael Makris,
  • Tarek Owaidah,
  • Michelle Sholzberg,
  • Andreas Tiede,
  • David J. Werring,
  • H. Bart van der Worp,
  • Jerzy Windyga,
  • Giancarlo Castaman

DOI
https://doi.org/10.1097/HS9.0000000000000900
Journal volume & issue
Vol. 7, no. 6
p. e900

Abstract

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Cardiovascular disease is an emerging medical issue in patients with hemophilia (PWH) and its prevalence is increasing up to 15% in PWH in the United States. Atrial fibrillation, acute and chronic coronary syndromes, venous thromboembolism, and cerebral thrombosis are frequent thrombotic or prothrombotic situations, which require a careful approach to fine-tune the delicate balance between thrombosis and hemostasis in PWH when using both procoagulant and anticoagulant treatments. Generally, PWH could be considered as being naturally anticoagulated when clotting factors are 20 IU/dL in need for any form of antithrombotic therapy, usually treatment without additional clotting factor prophylaxis could be used, but careful monitoring for bleeding is recommended. For antiplatelet treatment, this threshold could be lower with single-antiplatelet agent, but again factor level should be at least 20 IU/dL for dual antiplatelet treatment. In this complex growing scenario, the European Hematology Association in collaboration with the International Society on Thrombosis and Haemostasis, the European Association for Hemophilia and Allied Disorders, the European Stroke Organization, and a representative of the European Society of Cardiology Working Group on Thrombosis has produced this current guidance document to provide clinical practice recommendations for health care providers who care for PWH.