Recommendations

Blood Transfusion - 2 2020 (March-April)

Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab: AICE practical guidance in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET

Authors

Key words: haemophilia A, emicizumab, emergency, FVIII inhibitors, bypassing agents
Publication Date: 2019-10-18

Abstract

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agent. Given this, and in order to expand the current knowledge on the use of emicizumab and concomitant haemostatic agents, and reduce the risk of complications in this setting, the Italian Association of Haemophilia Centres (AICE) here provides guidance on the management of breakthrough bleeds and surgery in emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis. This paper has been shared with other National Scientific Societies involved in the field.

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Authors

Giancarlo Castaman - Centre for Bleeding Disorders and Coagulation, Department of Oncology, "Careggi" University Hospital, Florence

Cristina Santoro - Department of Haematology, "Policlinico Umberto I" University Hospital, Rome

Antonio Coppola - Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma

Maria E. Mancuso - "Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico", "Angelo Bianchi Bonomi" Haemophilia and Thrombosis Centre, Milan

Rita C. Santoro - Centre for Haemorrhagic and Thrombotic Disorders, "Pugliese Ciaccio" Hospital, Catanzaro

Sergio Bernardini - Department of Experimental Medicine and Surgery, University of Rome "Tor Vergata", Rome

Francesco R. Pugliese - Department of Emergency Medicine, "Sandro Pertini" Hospital, ASL Roma 2, Rome

Riccardo Lubrano - Department of Maternal and Child and Urological Sciences, Sapienza University of Rome, Rome

Maria Golato - Unit of Clinical Pathology, "SS Annunziata" University Hospital, ASL Lanciano-Vasto-Chieti, Chieti

Armando Tripodi - "Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico", "Angelo Bianchi Bonomi" Haemophilia and Thrombosis Centre, Milan; "Luigi Villa" Foundation, Milan

Angiola Rocino - Haemophilia and Thrombosis Centre, Haematology, S. Maria di Loreto Nuovo Hospital, ASL Napoli 1 Centro, Naples; Italy

Elena Santagostino - on behalf of the ad hoc Working Group; "Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico", "Angelo Bianchi Bonomi" Haemophilia and Thrombosis Centre, Milan

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