Sophie Susen1, Yves Gruel2, Anne Godier3,4, Annie Harroche5, Herve Chambost6, Dominique Lasne5, Antoine Rauch1, Stephanie Roullet7, Pierre Fontana8,9, Jenny Goudemand1, Emmanuel de Maistre10, Valerie Chamouard11, Bénédicte Wibaut1, Pierre Albaladejo12,13, Claude Négrier11. 1. CHU Lille, Université de Lille, Lille, France. 2. Département d'Hématologie-Hémostase, Hôpital Universitaire de Tours, Tours, France. 3. Service d'Anesthésie Réanimation, AP-HP, Hôpital Européen Georges Pompidou, Paris, France. 4. Inserm UMR-S1140, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 5. CRC MHC, Service d'Hématologie Clinique Hôpital Universitaire Necker Enfants Malades, Laboratoire d'Hématologie Générale, Hôpital Necker, AP-HP, Paris, France. 6. APHM, Hôpital d'Enfants La Timone, Service d'Hématologie Oncologie Pédiatrique et Aix Marseille Univ, INSERM, INRA, C2VN, Marseille, France. 7. Inserm U 1034, Service Anesthésie-Réanimation Pellegrin, Université de Bordeaux, CHU de Bordeaux, Bordeaux, France. 8. Service d'Angiologie et d'Hémostase, Département de Médecine, Hôpitaux Universitaires de Genève, Genève, Switzerland. 9. Geneva Platelet Group, Faculté de Médecine, Université de Genève, Genève, Switzerland. 10. Service d'Hématologie Biologique - Secteur Hémostase, Plateau Technique de Biologie, CHU Dijon-Bourgogne, Dijon, France. 11. Unité d'Hémostase Clinique, Louis Pradel Hospital, University Claude Bernard, Lyon, France. 12. Département d'Anesthésie-Réanimation, CHU Grenoble-Alpes, Grenoble, France. 13. ThEMAS, TIMC, UMR CNRS 5525, Université Grenoble-Alpes, Grenoble, France.
Abstract
INTRODUCTION: Emicizumab (Hemlibra® ) recently became available and requires an adaptation for managing bleeding, suspected bleeding and emergency or scheduled invasive procedures in haemophilia A patients with inhibitor. This implicates a multidisciplinary approach and redaction of recommendations for care that must be regularly adapted to the available data. AIM: The following text aims to provide a guide for the management of people with haemophilia A with inhibitor treated with emicizumab in case of bleeding or invasives procedures. METHODS: The French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia (CRH), in collaboration with the French Working Group on Perioperative Haemostasis (GIHP) have been working together to make proposals for the management of these situations. RESULTS: Haemostatic treatment and other medications should be given stepwise, according to the severity and location of the bleeding or the risk of bleeding of the procedure as well as the haemostatic response obtained at each step in order to ensure an optimal benefit/risk ratio. CONCLUSION: The lack of data means that it is only possible to issue proposals rather than recommendations.
INTRODUCTION:Emicizumab (Hemlibra® ) recently became available and requires an adaptation for managing bleeding, suspected bleeding and emergency or scheduled invasive procedures in haemophilia Apatients with inhibitor. This implicates a multidisciplinary approach and redaction of recommendations for care that must be regularly adapted to the available data. AIM: The following text aims to provide a guide for the management of people with haemophilia A with inhibitor treated with emicizumab in case of bleeding or invasives procedures. METHODS: The French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia (CRH), in collaboration with the French Working Group on Perioperative Haemostasis (GIHP) have been working together to make proposals for the management of these situations. RESULTS: Haemostatic treatment and other medications should be given stepwise, according to the severity and location of the bleeding or the risk of bleeding of the procedure as well as the haemostatic response obtained at each step in order to ensure an optimal benefit/risk ratio. CONCLUSION: The lack of data means that it is only possible to issue proposals rather than recommendations.
Authors: Giancarlo Castaman; Cristina Santoro; Antonio Coppola; Maria E Mancuso; Rita C Santoro; Sergio Bernardini; Francesco R Pugliese; Riccardo Lubrano; Maria Golato; Armando Tripodi; Angiola Rocino; Elena Santagostino; Chiara Biasoli; Alessandra Borchiellini; Alberto Catalano; Laura Contino; Antonella Coluccia; Dorina Cultrera; Raimondo De Cristofaro; Giovanni Di Minno; Andrea Fabbri; Massimo Franchini; Gabriella Gamba; Anna Chiara Giuffrida; Paolo Gresele; Adele Giampaolo; Hamisa J Hassan; Matteo Luciani; Emanuela Marchesini; Renato Marino; Maria Gabriella Mazzucconi; Angelo C Molinari; Massimo Morfini; Lucia D Notarangelo; Lucia Peccarisi; Flora Peyvandi; Berardino Pollio; Gianna Franca Rivolta; Maria Pia Ruggieri; Vittorio Sargentini; Mario Schiavoni; Laura Sciacovelli; Maria Luisa Serino; Sergio Siragusa; Annarita Tagliaferri; Sophie Testa; Alberto Tosetto; Stefania Zampogna; Ezio Zanon Journal: Blood Transfus Date: 2019-10-18 Impact factor: 3.443
Authors: C Hermans; P L F Giangrande; B O'Mahony; P de Kleijn; M Bedford; A Batorova; J Blatný; K Jansone Journal: Orphanet J Rare Dis Date: 2020-08-24 Impact factor: 4.123
Authors: Hans H Brackmann; Wolfgang Schramm; Johannes Oldenburg; Viridiana Cano; Peter L Turecek; Claude Négrier Journal: Hamostaseologie Date: 2020-07-27 Impact factor: 2.145