Management of acquired hemophilia a: results from the spanish registry.

Show simple item record María-Eva Mingot-Castellano Josep Pardos-Gea Saturnino Haya José-Marı a Bastida-Bermejo Dolors Tàssies Ana Marco-Rico Ramiro Nuñez Faustino García-Candel María-Carmen Fernández-Sánchez de Mora Inmaculada Soto María-Teresa Álvarez-Román Susana Asenjo Marina Carrasco Rafael Lluch-García José-Manuel Martín-Antorán Agustín Rodríguez-Alén Elena Roselló Laura Torres-Miñana Shally Marcellini-Antonio Ana Moretó-Quinana José-Antonio Rodríguez-García Reyes Aguinaco-Culebras Nieves Alonso-Escobar Carlos Cervero-Santiago Núria Fernández-Mosteirín María-Paz Martínez-Badás Montserrat Pérez-Sánchez Rocío Pérez-Montes Ramón Rodríguez-González Marisol Uribe-Barrientos Isabel Socorro Caparrós-Miranda Miriam Iglesias-Fernández Ángela Baena Manuel Rodríguez-López Ana Sebrango-Sandia Irene Vázquez-Fernández Pascual Marco 2022-12-14T09:42:21Z 2022-12-14T09:42:21Z
dc.description.abstract [eng] The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.
dc.format application/pdf
dc.relation.ispartof Blood Advances, 2021, vol. 5, num. 19, p. 3821-3829
dc.rights , 2021
dc.subject.classification 57 - Biologia
dc.subject.classification Ciències de la salut
dc.subject.other 57 - Biological sciences in general
dc.subject.other Medical sciences
dc.title Management of acquired hemophilia a: results from the spanish registry.
dc.type info:eu-repo/semantics/article 2022-12-14T09:42:21Z
dc.rights.accessRights info:eu-repo/semantics/openAccess

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