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Title: Management of acquired hemophilia A: results from the Spanish registry
Author: Mingot Castellano, María Eva
Pardos Gea, Josep
Haya, Saturnino
Bastida Bermejo, José María
Tàssies, Dolors
Marco Rico, Ana
Núñez, Ramiro
García Candel, Faustino
Fernández Sánchez de Mora, María Carmen
Soto, Inmaculada
Álvarez Román, María Teresa
Asenjo, Susana
Carrasco, Marina (Carrasco Pérez)
Lluch García, Rafael
Martín Antorán, José Manuel
Rodríguez Alén, Agustín
Roselló, Elena
Torres Miñana, Laura
Marcellini Antonio, Shally
Moretó Quinana, Ana
Rodríguez García, José Antonio
Aguinaco Culebras, Reyes
Alonso Escobar, Nieves
Cervero Santiago, Carlos
Fernández Mosteirín, Núria
Martínez Badás, María Paz
Pérez Sánchez, Montserrat
Pérez Montes, Rocío
Rodríguez González, Ramón
Uribe Barrientos, Marisol
Caparrós Miranda, Isabel Socorro
Iglesias Fernández, Miriam
Baena, Ángela
Rodríguez López, Manuel
Sebrango Sandia, Ana
Vázquez Fernández, Irene
Marco, Pascual
Spanish Society of Thrombosis and Haemostasis (SETH)
Keywords: Hemofília
Issue Date: 14-Sep-2021
Publisher: American Society of Hematology
Abstract: 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.
Note: Reproducció del document publicat a:
It is part of: Blood Advances, 2021, vol. 5, num. 19, p. 3821-3829
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ISSN: 2473-9537
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

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