| Title: |
Management of acquired hemophilia A: results from the Spanish registry |
| Authors: |
Mingot Castellano, María Eva; Pardos Gea, Josep; Haya, Saturnino; Bastida Bermejo, José María; Tàssies Penella, María Dolores; 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) |
| Source: |
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
| Publisher Information: |
American Society of Hematology |
| Publication Year: |
2021 |
| Collection: |
Dipòsit Digital de la Universitat de Barcelona |
| Subject Terms: |
Hemofília; Espanya; Hemophilia; Spain |
| Description: |
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. |
| Document Type: |
article in journal/newspaper |
| File Description: |
9 p.; application/pdf |
| Language: |
English |
| Relation: |
Reproducció del document publicat a: https://doi.org/10.1182/bloodadvances.2021004626; Blood Advances, 2021, vol. 5, num. 19, p. 3821-3829; https://doi.org/10.1182/bloodadvances.2021004626; https://hdl.handle.net/2445/180958 |
| Availability: |
https://hdl.handle.net/2445/180958 |
| Rights: |
(c) American Society of Hematology, 2021 ; info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.B25ED8FE |
| Database: |
BASE |