TY - JOUR
T1 - Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding
AU - Coppola, Antonio
AU - Franchini, Massimo
AU - Tripodi, Armando
AU - Santoro, Rita C
AU - Castaman, Giancarlo
AU - Marino, Renato
AU - Zanon, Ezio
AU - Santoro, Cristina
AU - Rivolta, Gianna F
AU - Contino, Laura
AU - De Cristofaro, Raimondo
AU - Molinari, Angelo C
AU - Gresele, Paolo
AU - Rocino, Angiola
PY - 2022
Y1 - 2022
N2 - Background - Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies to coagulation factor VIII that may be secondary to autoimmune diseases, cancer, drugs, pregnancy, infections, or be idiopathic. Recurrent bleeding, often severe, mostly in muscles and soft tissues, and isolated prolonged activated partial thromboplastin time (aPTT), in the absence of personal and family history of bleeding, are typical features that should raise the suspicion of AHA. Poor awareness of the disease results in diagnostic delays and inappropriate treatment. Materials and methods - The Italian Association of Haemophilia Centres (AICE) developed consensus recommendations in cooperation with the Italian Society on Thrombosis and Haemostasis (SISET). The document was shared with scientific societies of specialist physicians, laboratory professionals and pharmacists to spread knowledge about AHA and promote appropriate diagnosis/treatment. Results - Ready availability of the aPTT mixing test is crucial, although diagnostic confirmation and optimal management require prompt referral of patients to specialised centres with rapidly available diagnostic and therapeutic facilities. If immediate referral is unfeasible, treatment must be undertaken early, under guidance of specialised centres or based on shared protocols. Recommendations about diagnosis, general management and, in bleeding patients, haemostatic therapy using bypassing agents or replacement treatment, including the recently available recombinant porcine factor VIII, are provided, considering the different clinical settings and laboratory facilities. Discussion - This consensus document aims to improve the overall healthcare pathways for AHA, harmonise the management and therapeutic approaches to newly diagnosed patients and reduce the still relevant complications and mortality in this setting.
AB - Background - Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies to coagulation factor VIII that may be secondary to autoimmune diseases, cancer, drugs, pregnancy, infections, or be idiopathic. Recurrent bleeding, often severe, mostly in muscles and soft tissues, and isolated prolonged activated partial thromboplastin time (aPTT), in the absence of personal and family history of bleeding, are typical features that should raise the suspicion of AHA. Poor awareness of the disease results in diagnostic delays and inappropriate treatment. Materials and methods - The Italian Association of Haemophilia Centres (AICE) developed consensus recommendations in cooperation with the Italian Society on Thrombosis and Haemostasis (SISET). The document was shared with scientific societies of specialist physicians, laboratory professionals and pharmacists to spread knowledge about AHA and promote appropriate diagnosis/treatment. Results - Ready availability of the aPTT mixing test is crucial, although diagnostic confirmation and optimal management require prompt referral of patients to specialised centres with rapidly available diagnostic and therapeutic facilities. If immediate referral is unfeasible, treatment must be undertaken early, under guidance of specialised centres or based on shared protocols. Recommendations about diagnosis, general management and, in bleeding patients, haemostatic therapy using bypassing agents or replacement treatment, including the recently available recombinant porcine factor VIII, are provided, considering the different clinical settings and laboratory facilities. Discussion - This consensus document aims to improve the overall healthcare pathways for AHA, harmonise the management and therapeutic approaches to newly diagnosed patients and reduce the still relevant complications and mortality in this setting.
KW - acquired haemophilia A
KW - bleeding
KW - inhibitors
KW - haemostatic treatment
KW - coagulation tests
KW - acquired haemophilia A
KW - bleeding
KW - inhibitors
KW - haemostatic treatment
KW - coagulation tests
UR - http://hdl.handle.net/10807/303911
U2 - 10.2450/2022.0238-21
DO - 10.2450/2022.0238-21
M3 - Article
SN - 2385-2070
VL - 20
SP - 245
EP - 262
JO - BLOOD TRANSFUSION
JF - BLOOD TRANSFUSION
ER -