RT Journal Article
T1 Management of acquired hemophilia A: results from the Spanish registry.
A1 Mingot-Castellano, María-Eva
A1 Pardos-Gea, Josep
A1 Haya, Saturnino
A1 Bastida-Bermejo, José-María
A1 Tàssies, Dolors
A1 Marco-Rico, Ana
A1 Núñez, Ramiro
A1 García-Candel, Faustino
A1 de Mora, María-Carmen Fernández-Sanchez
A1 Soto, Inmaculada
A1 Álvarez-Román, María-Teresa
A1 Asenjo, Susana
A1 Carrasco, Marina
A1 Lluch-García, Rafael
A1 Martín-Antorán, José-Manuel
A1 Rodríguez-Alén, Agustín
A1 Roselló, Elena
A1 Torres-Miñana, Laura
A1 Marcellini-Antonio, Shally
A1 Moretó-Quinana, Ana
A1 Rodríguez-García, José-Antonio
A1 Aguinaco-Culebras, Reyes
A1 Alonso-Escobar, Nieves
A1 Cervero-Santiago, Carlos
A1 Fernández-Mosteirín, Núria
A1 Martínez-Badás, María-Paz
A1 Pérez-Sánchez, Montserrat
A1 Pérez-Montes, Rocío
A1 Rodríguez-González, Ramón
A1 Uribe-Barrientos, Marisol
A1 Caparrós-Miranda, Isabel Socorro
A1 Iglesias-Fernández, Miriam
A1 Baena, Ángela
A1 Rodríguez-López, Manuel
A1 Sebrango-Sandia, Ana
A1 Vázquez-Fernández, Irene
A1 Marco, Pascual
A1 Acquired Haemophilia Spanish Registry of the Spanish Society of ThrombosisHaemostasis (SETH), 
AB 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.
YR 2021
FD 2021
LK https://hdl.handle.net/10668/25917
UL https://hdl.handle.net/10668/25917
LA en
DS RISalud
RD Apr 7, 2025