title: Acquired haemophilia: Epidemiology, clinical presentation, diagnosis and treatment.
abstract: The development of circulating autoantibodies able to inhibit some coagulation proteins induces severe or even life-threatening bleeding. This disorder is called acquired haemophilia. This is a rare disease, although its impact may be underestimated because of the lack of records, the lack of knowledge by many specialists, the complexity of the laboratory diagnosis and, finally, because of the fulminant clinical presentation that often precludes diagnosis. Several studies established that mortality ranges between 9 and 33%. Not only haematologists but all physicians should be trained to follow the right steps to diagnose these patients as soon as possible in order to reduce such mortality rates. This review approaches the basic concepts dealing with the diagnosis and management of these patients and intends to assist physicians in identifying patients under suspicion of acquired haemophilia to correctly manage them and refer them to the appropriate Haemostasis Unit.
authors.affiliation: Unidad de Gestión Clínica Intercentros de Hematología, Hospital Regional Universitario de Málaga, Málaga, España. Electronic address: mariae.mingot.sspa@juntadeandalucia.es.
authors.forename: Maria Eva
authors.initials: ME
authors.lastname: Mingot-Castellano
authors.affiliation: Unidad de Gestión Clínica Intercentros de Hematología, Hospital Universitario Virgen Macarena y Hospital Universitario Virgen del Rocío, IBIS, Sevilla, España.
authors.forename: Ramiro
authors.initials: R
authors.lastname: Núñez
authors.affiliation: Unidad de Gestión Clínica Intercentros de Hematología, Hospital Universitario Virgen Macarena y Hospital Universitario Virgen del Rocío, IBIS, Sevilla, España.
authors.forename: Francisco Javier
authors.initials: FJ
authors.lastname: Rodríguez-Martorell
mesh_terms: D015415:Biomarkers / Q000097:blood; D004359:Drug Therapy, Combination; D006401:Hematologic Agents / Q000627:therapeutic use; D006467:Hemophilia A / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010956:Plasmapheresis; D011379:Prognosis
chemical_list: D015415:Biomarkers; D006401:Hematologic Agents; D007166:Immunosuppressive Agents
keywords: Acquired haemophilia; Anti-Factor VIII autoantibodies; Antihaemorragic treatment; Autoanticuerpos antifactor viii; Eradication of factor VIII Inhibitor; Erradicación del inhibidor del factor viii; Factor VIII; Factor VIII Inhibitor; Hemofilia adquirida; Inhibidor del factor viii; Tratamiento antihemorrágico