TY - JOUR
T1 - Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab
T2 - AICE practical guidance in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET
AU - ad hoc Working Group
AU - Castaman, Giancarlo
AU - Santoro, Cristina
AU - Coppola, Antonio
AU - Mancuso, Maria E.
AU - Santoro, Rita C.
AU - Bernardini, Sergio
AU - Pugliese, Francesco R.
AU - Lubrano, Riccardo
AU - Golato, Maria
AU - Tripodi, Armando
AU - Rocino, Angiola
AU - Santagostino, Elena
AU - Biasoli, Chiara
AU - Borchiellini, Alessandra
AU - Catalano, Alberto
AU - Contino, Laura
AU - Coluccia, Antonella
AU - Cultrera, Dorina
AU - De Cristofaro, Raimondo
AU - Di Minno, Giovanni
AU - Fabbri, Andrea
AU - Franchini, Massimo
AU - Gamba, Gabriella
AU - Giuffrida, Anna Chiara
AU - Gresele, Paolo
AU - Giampaolo, Adele
AU - Hassan, Hamisa J.
AU - Luciani, Matteo
AU - Marchesini, Emanuela
AU - Marino, Renato
AU - Mazzucconi, Maria Gabriella
AU - Molinari, Angelo C.
AU - Morfini, Massimo
AU - Notarangelo, Lucia D.
AU - Peccarisi, Lucia
AU - Peyvandi, Flora
AU - Pollio, Berardino
AU - Rivolta, Gianna Franca
AU - Ruggieri, Maria Pia
AU - Sargentini, Vittorio
AU - Schiavoni, Mario
AU - Sciacovelli, Laura
AU - Serino, Maria Luisa
AU - Siragusa, Sergio
AU - Tagliaferri, Annarita
AU - Testa, Sophie
AU - Tosetto, Alberto
AU - Zampogna, Stefania
AU - Zanon, Ezio
PY - 2020/3/1
Y1 - 2020/3/1
N2 - 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.
AB - 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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U2 - 10.2450/2019.0186-19
DO - 10.2450/2019.0186-19
M3 - Article
C2 - 31657709
VL - 18
SP - 143
EP - 151
JO - Blood Transfusion
JF - Blood Transfusion
SN - 1723-2007
IS - 2
ER -