Antonio Coppola1, Massimo Franchini2, Armando Tripodi3, Rita C Santoro4, Giancarlo Castaman5, Renato Marino6, Ezio Zanon7, Cristina Santoro8, Gianna F Rivolta1, Laura Contino9, Raimondo De Cristofaro10, Angelo C Molinari11, Paolo Gresele12, Angiola Rocino13. 1. Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy. 2. Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy. 3. Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, IRCCS Ca' Granda Maggiore Hospital Foundation, and Luigi Villa Foundation, Milan, Italy. 4. Centre for Haemorrhagic and Thrombotic Disorders, Pugliese Ciaccio Hospital, Catanzaro, Italy. 5. Centre for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, Florence, Italy. 6. Haemophilia and Thrombosis Centre, University Hospital of Bari, Bari, Italy. 7. Haemophilia Centre, University Hospital of Padua, Padua, Italy. 8. Haematology, Umberto I University Hospital, Rome, Italy. 9. SS. Antonio e Biagio Hospital, Alessandria, Italy. 10. Centre for Haemorrhagic and Thrombotic Diseases, IRCCS A. Gemelli University Hospital Foundation, and Department of Translational Medicine, Sacro Cuore Catholic University, Rome, Italy. 11. Regional Reference Centre for Haemorrhagic Diseases, Thrombosis and Haemostasis Unit, IRCCS Giannina Gaslini Hospital, Genoa, Italy. 12. Section of Internal and Cardiovascular Medicine, Department of Medicine and Surgery, University of Perugia, Perugia, Italy. 13. Haemophilia and Thrombosis Centre, Haematology, Ospedale del Mare, ASL Napoli 1 Centro, Naples, Italy.
Abstract
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.
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.
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