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

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, but specific recommendations in patients with very low levels according to the different clinical situations are lacking and mainly based on the anecdotal series. For PWH with baseline clotting factor levels >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.
Competing Interests: RS: scientific editor of HemaSphere. Unrestricted grants or consultancy fees from Bayer, CSL Behring, Hemab, NovoNordisk, Octapharma, Sanofi, Sobi, Takeda [all fees to the institution]. VJ-Y: Consultancy: Pfizer, Shire, NovoNordisk, Sobi, Grifols, Roche, CSL Behring; Research funding: Pfizer, Shire, NovoNordisk, Sobi, Octapharma, Roche, Grifols, CSL Behring; Membership on an entity’s board of directors, speaker’s bureau, or its advisory committees: NovoNordisk, Pfizer, Shire, Sobi, Grifols, Roche, CSL Behring. MAE: Consultancy fees from NovoNordisk, BioMarin, CSL Behring, Genentech/Roche, Sanofi, Takeda, Pfizer, Bayer, Hemobiologics/LFB, Kedrion, UniQure. Participated in clinical trials sponsored by: NovoNordisk, UniQure, Takeda, Bayer, Genentech/Roche, OPKO Biologics, CSL Behring. AF: honoraria for lectures from Sanofi, Stago, Bayer, and Pfizer. RK: Research grants: Bayer, CSL Behring, LEO, NovoNordisk. Consultancy and speaker fees from BioMarin, Bayer, Biotest, Chugai, CSL Behring, Daiichi Sankyo, Grifols, NovoNordisk, Octapharma, Pfizer, Roche, Sanofi, SOBI, Takeda. RL: Consultancy fees from Alexion, Astra Zeneca, Bayer, BioMarin, CSL Behring, Novo Nordisk, Pfizer, Roche, Sanofi, SOBI, Takeda. FWGL: Unrestricted grants/research funding from CSL Behring, UniQure, Sobi, Takeda; consultancy fees from BioMarin, CSL Behring, Takeda, and uniQure [all fees to the institution], and served as DSMB member for a study sponsored by Roche. MM: Consultancy fees from NovoNordisk, Grifols, Sanofi, Freeline, Biotest. He is the project lead for the European Hemophilia Safety Surveillance (EUHASS) safety reporting scheme which is funded by Bayer, BioMarin, BPL, CSL Behring, Grifols, Kedrion, NovoNordisk, Octapharma, Roche, Sanofi, Sobi, and Takeda. TO: consulting fees from Bayer, Sobi and Novo Nordisk. MS: Unrestricted research funding from Octapharma, Pfizer and Amgen (to institution); honoraria from advisory boards from Amgen, Novartis, Octapharma, Pfizer, and Amgen. AT: grants and personal fees for lectures and consultancy from Bayer, BioMarin, Biotest, Chugai, CSL Behring, Novo Nordisk, Octapharma, Pfizer, Roche, SOBI, and Takeda. DW: has received: grant funding from the Stroke Association and British Heart Foundation; speaking honoraria from Bayer; speaking and chairing honoraria from Alexion and NovoNordisk; and consultancy fees from Bayer and NovoNordisk. HBvdW: funding for consultancy from Bayer and TargED, all paid to institution. JW: Research support and honoraria for lectures from: Alnylam, Amgen, Bayer, CSL Behring, LFB, Novartis, Novo Nordisk, Octapharma, Roche, Sanofi, Siemens, Sobi, Swixx Biopharma, Takeda. GC: Unrestricted grants/research funding from CSL Behring, Sobi, Pfizer; speaker/consultancy fees from Bayer, BioMarin, CSL Behring, Grifols, Kedrion, LFB, NovoNordisk, Roche, Sanofi, Takeda, uniQure and Werfen. All the other authors have no conflicts of interest to disclose.
(Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.)