Pierre Fontana1, Lorenzo Alberio2, Manuela Albisetti3, Anne Angelillo-Scherrer4, Lars M Asmis5, Alessandro Casini1, Bernhard Gerber6, Lukas Graf, Inga Hegemann7, Wolfgang Korte7, Maria Martinez8, Jan-Dirk Studt9, Dimitrios A Tsakiris10, Walter A Wuillemin11, Johanna A Kremer Hovinga4. 1. Division of Angiology and Haemostasis, University Hospitals of Geneva, Switzerland. 2. Service and Central Laboratory of Haematology, Lausanne University Hospital, Lausanne, Switzerland. 3. Division of Haematology, University Children's Hospital, Zurich, Switzerland. 4. Department of Haematology and Central Haematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Switzerland. 5. Centre for perioperative Thrombosis and Haemostasis, Zurich, Switzerland. 6. Clinic of Haematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland. 7. Cantonal Hospital of St Gallen, Switzerland. 8. Dagnostic Haematology, Basel University Hospital, Basel, Switzerland. 9. Department of Medical Oncology and Haematology, University Hospital Zurich, Switzerland. 10. Diagnostic Haematology, Basel University Hospital, Basel, Switzerland. 11. Division of Hematology and Central Hematology Laboratory, Cantonal Hospital of Lucerne, Lucerne, and University of Bern, Switzerland.
Abstract
INTRODUCTION: Emicizumab (Hemlibra®, Hoffmann-La Roche, Switzerland) is now available for haemophilia A patients with or without factor VIII inhibitors. Management of bleeding events and replacement therapy for invasive procedures have to be adapted. OBJECTIVE: To provide a practical guidance for the management of breakthrough bleeding events and elective or urgent surgery in adult and paediatric patients with haemophilia A without inhibitors treated with emicizumab. METHODS: Based on the available literature and the experiences collected from adult and paediatric patients treated in Switzerland, the Working Party on Haemostasis of the Swiss Society of Haematology and the Swiss Haemophilia Network worked together to reach a consensus on the management of bleeding events and invasive procedures. RESULTS AND CONCLUSION: Minor bleeding events and invasive procedures associated with low bleeding risk can be treated without factor replacement therapy in most cases, whereas major bleeding events and high-risk surgery require additional factor VIII replacement at usual doses, at least for the first days. Emicizumab treatment should be continued throughout the procedure and during the postoperative period. Elective major surgery should be planned according to emicizumab dosing for patients with a once-a-month posology. Of note, so far only few data are available on the management of major bleeds and surgery in patients with haemophilia A treated with emicizumab and this practical guidance will have to be regularly updated with growing experience.  .
INTRODUCTION:Emicizumab (Hemlibra®, Hoffmann-La Roche, Switzerland) is now available for haemophilia Apatients with or without factor VIII inhibitors. Management of bleeding events and replacement therapy for invasive procedures have to be adapted. OBJECTIVE: To provide a practical guidance for the management of breakthrough bleeding events and elective or urgent surgery in adult and paediatric patients with haemophilia A without inhibitors treated with emicizumab. METHODS: Based on the available literature and the experiences collected from adult and paediatric patients treated in Switzerland, the Working Party on Haemostasis of the Swiss Society of Haematology and the Swiss Haemophilia Network worked together to reach a consensus on the management of bleeding events and invasive procedures. RESULTS AND CONCLUSION: Minor bleeding events and invasive procedures associated with low bleeding risk can be treated without factor replacement therapy in most cases, whereas major bleeding events and high-risk surgery require additional factor VIII replacement at usual doses, at least for the first days. Emicizumab treatment should be continued throughout the procedure and during the postoperative period. Elective major surgery should be planned according to emicizumab dosing for patients with a once-a-month posology. Of note, so far only few data are available on the management of major bleeds and surgery in patients with haemophilia A treated with emicizumab and this practical guidance will have to be regularly updated with growing experience.  .
Authors: Anouk A M T Donners; László Gerencsér; Kim C M van der Elst; Toine C G Egberts; Moniek P M de Maat; Albert Huisman; Rolf T Urbanus; Mohsin El Amrani Journal: Res Pract Thromb Haemost Date: 2022-06-08