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
Affiliations
Roger E.G. Schutgens
1 Center for Benign Hematology, Thrombosis and Hemostasis, Van Creveldkliniek University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
Victor Jimenez-Yuste
2 Hematology Department, La Paz University Hospital, Autonoma University, Madrid, Spain
Miguel Escobar
3 University of Texas Health Science Center at Houston, TX, USA
Anna Falanga
4 University of Milano Bicocca, School of Medicine, Monza, Italy
Bruna Gigante
6 Division of Cardiovascular Medicine, Department of Medicine, Karolinska Institutet, Solna, Sweden
Robert Klamroth
8 Department of Internal Medicine Angiology and Coagulation Disorders at the Vivantes Klinikum im Friedrichshain, Berlin, Germany
Riitta Lassila
10 Department of Hematology, Coagulation Disorders Unit, and Research Program Unit in Systems Oncology Oncosys, Medical Faculty, University of Helsinki, Finland
Frank W.G. Leebeek
11 Department of Hematology, Erasmus MC, University Medical Center Rotterdam, The Netherlands
Michael Makris
12 Haemophilia and Thrombosis Centre, University of Sheffield, United Kingdom
Tarek Owaidah
13 King Faisal Specialist Hospital and Research Centre, Alfaisal University, Riyadh, Saudi Arabia
Michelle Sholzberg
14 Division of Hematology-Oncology, Departments of Medicine, and Laboratory Medicine and Pathobiology, St. Michael’s Hospital, Li Ka Shing Knowledge Institute, University of Toronto, Canada
Andreas Tiede
15 Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Germany
David J. Werring
16 Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, London, United Kingdom
H. Bart van der Worp
17 University Medical Center (UMC), Utrecht University, The Netherlands
Jerzy Windyga
18 Department of Hemostasis Disorders and Internal Medicine, Laboratory of Hemostasis and Metabolic Diseases, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
Giancarlo Castaman
19 Center for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, Florence, Italy
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.
