Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab

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...

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Main Authors: Pierre Fontana, Lorenzo Alberio, Manuela Albisetti, Anne Angelillo-Scherrer, Lars M. Asmis, Alessandro Casini, Bernhard Gerber, Lukas Graf, Inga Hegemann, Wolfgang Korte, Maria Martinez, Jan-Dirk Studt, Dimitrios A. Tsakiris, Walter A. Wuillemin, Johanna A. Kremer Hovinga
Format: Article
Language:English
Published: SMW supporting association (Trägerverein Swiss Medical Weekly SMW) 2020-12-01
Series:Swiss Medical Weekly
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Online Access:https://www.smw.ch/index.php/smw/article/view/2933
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author Pierre Fontana
Lorenzo Alberio
Manuela Albisetti
Anne Angelillo-Scherrer
Lars M. Asmis
Alessandro Casini
Bernhard Gerber
Lukas Graf
Inga Hegemann
Wolfgang Korte
Maria Martinez
Jan-Dirk Studt
Dimitrios A. Tsakiris
Walter A. Wuillemin
Johanna A. Kremer Hovinga
author_facet Pierre Fontana
Lorenzo Alberio
Manuela Albisetti
Anne Angelillo-Scherrer
Lars M. Asmis
Alessandro Casini
Bernhard Gerber
Lukas Graf
Inga Hegemann
Wolfgang Korte
Maria Martinez
Jan-Dirk Studt
Dimitrios A. Tsakiris
Walter A. Wuillemin
Johanna A. Kremer Hovinga
author_sort Pierre Fontana
collection DOAJ
description 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.
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spelling doaj.art-c631ea6a400c4eddaa79cbb79fb46bd92024-11-02T11:59:34ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972020-12-01150515310.4414/smw.2020.20422Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumabPierre Fontana0Lorenzo Alberio1Manuela Albisetti2Anne Angelillo-Scherrer3Lars M. Asmis4Alessandro Casini5Bernhard Gerber6Lukas Graf7Inga Hegemann8Wolfgang Korte9Maria Martinez10Jan-Dirk Studt11Dimitrios A. Tsakiris12Walter A. Wuillemin13Johanna A. Kremer Hovinga14Division of Angiology and Haemostasis, University Hospitals of Geneva, SwitzerlandService and Central Laboratory of Haematology, Lausanne University Hospital, Lausanne, SwitzerlandDivision of Haematology, University Children’s Hospital, Zurich, SwitzerlandDepartment of Haematology and Central Haematology Laboratory, Inselspital, Bern University Hospital, University of Bern, SwitzerlandCentre for perioperative Thrombosis and Haemostasis, Zurich, SwitzerlandDivision of Angiology and Haemostasis, University Hospitals of Geneva, SwitzerlandClinic of Haematology, Oncology Institute of Southern Switzerland, Bellinzona, SwitzerlandCantonal Hospital of St Gallen, SwitzerlandDepartment of Medical Oncology and Haematology, University Hospital Zurich, SwitzerlandCantonal Hospital of St Gallen, SwitzerlandDiagnostic Haematology, Basel University Hospital, Basel, SwitzerlandDepartment of Medical Oncology and Haematology, University Hospital Zurich, SwitzerlandDiagnostic Haematology, Basel University Hospital, Basel, SwitzerlandDivision of Hematology and Central Hematology Laboratory, Cantonal Hospital of Lucerne, Lucerne, and University of Bern, SwitzerlandDepartment of Haematology and Central Haematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Switzerland 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. https://www.smw.ch/index.php/smw/article/view/2933emicizumabbleedsinvasive proceduresGuidelines
spellingShingle Pierre Fontana
Lorenzo Alberio
Manuela Albisetti
Anne Angelillo-Scherrer
Lars M. Asmis
Alessandro Casini
Bernhard Gerber
Lukas Graf
Inga Hegemann
Wolfgang Korte
Maria Martinez
Jan-Dirk Studt
Dimitrios A. Tsakiris
Walter A. Wuillemin
Johanna A. Kremer Hovinga
Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
Swiss Medical Weekly
emicizumab
bleeds
invasive procedures
Guidelines
title Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
title_full Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
title_fullStr Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
title_full_unstemmed Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
title_short Management of bleeding events and invasive procedures in patients with haemophilia A without inhibitors treated with emicizumab
title_sort management of bleeding events and invasive procedures in patients with haemophilia a without inhibitors treated with emicizumab
topic emicizumab
bleeds
invasive procedures
Guidelines
url https://www.smw.ch/index.php/smw/article/view/2933
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