Blood Transfusion - 3 2022 (May-June)

Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding


Key words: acquired haemophilia A, bleeding, coagulation tests, haemostatic treatment, inhibitors
Publication Date: 2022-01-20


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.



Antonio Coppola - Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy; 2Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy

Massimo Franchini - Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy

Armando Tripodi - Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, IRCCS Ca’ Granda Maggiore Hospital Foundation, and Luigi Villa Foundation, Milan, Italy

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

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

Renato Marino - Haemophilia and Thrombosis Centre, University Hospital of Bari, Bari, Italy

Ezio Zanon - Haemophilia Centre, University Hospital of Padua, Padua, Italy

Cristina Santoro - Haematology, Umberto I University Hospital, Rome, Italy

Gianna F. Rivolta - Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy

Laura Contino - SS. Antonio e Biagio Hospital, Alessandria, Italy

Raimondo De Cristofaro - Centre for Haemorrhagic and Thrombotic Diseases, IRCCS A. Gemelli University Hospital Foundation, and Department of Translational Medicine, Sacro Cuore Catholic University, Rome, Italy

Angelo C. Molinari - Regional Reference Centre for Haemorrhagic Diseases, Thrombosis and Haemostasis Unit, IRCCS Giannina Gaslini Hospital, Genoa, Italy

Paolo Gresele - Section of Internal and Cardiovascular Medicine, Department of Medicine and Surgery, University of Perugia, Perugia, Italy

Angiola Rocino - Haemophilia and Thrombosis Centre, Haematology, Ospedale del Mare, ASL Napoli 1 Centro, Naples, Italy

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