title: Acquired haemophilia A: a 2013 update.
abstract: Acquired haemophilia A (AHA) is a rare but often severe bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). AHA occurs more frequently in the elderly and in association with several conditions, such as the post-partum period, malignancies, autoimmune diseases or drug exposure; however, approximately 50% of reported cases are apparently idiopathic. Beside the elimination of the underlying disorder, the therapeutic approach to AHA should be directed toward the control of acute bleed and the eradication of FVIII autoantibody production. In this narrative review, we summarise the current knowledge on the epidemiology, diagnosis and clinical features of AHA, focusing in particular on advances in the management of this challenging bleeding disorder.
authors.affiliation: Pier Mannuccio Mannucci, MD, Scientific Direction, IRCCS Cà Granda Foundation Maggiore Policlinico Hospital, Via Pace 9, 20122 Milan, Italy, Tel.: +39 02 5503 5414, Fax: +39 02 54 100 125, E-mail: piermannuccio.mannucci@policlinico.mi.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.forename: Pier Mannuccio
authors.initials: PM
authors.lastname: Mannucci
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000378:metabolism; D001324:Autoantigens / Q000276:immunology*; D015897:Comorbidity; D005169:Factor VIII / Q000276:immunology*; D006467:Hemophilia A / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology; D006489:Hemostatic Techniques; D006801:Humans
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D005169:Factor VIII
keywords: Acquired haemophilia A; FVIII; auto-antibodies; bleeding; inhibitors; therapy