Management of acquired hemophilia A: results from the Spanish registry

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)

Management of acquired hemophilia A: results from the Spanish registry

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 mor...

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Autores:	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)
Tipo de recurso:	artículo
Estado:	Versión publicada
Fecha de publicación:	2021
País:	España
Institución:	Universidad de Barcelona
Repositorio:	Dipòsit Digital de la UB
OAI Identifier:	oai:diposit.ub.edu:2445/180958
Acceso en línea:	https://hdl.handle.net/2445/180958
Access Level:	acceso abierto
Palabra clave:	Hemofília Espanya Hemophilia Spain

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spelling	Management of acquired hemophilia A: results from the Spanish registryMingot Castellano, María EvaPardos Gea, JosepHaya, SaturninoBastida Bermejo, José MaríaTàssies Penella, María DoloresMarco Rico, AnaNúñez, RamiroGarcía Candel, FaustinoFernández Sánchez de Mora, María CarmenSoto, InmaculadaÁlvarez Román, María TeresaAsenjo, SusanaCarrasco, Marina (Carrasco Pérez)Lluch García, RafaelMartín Antorán, José ManuelRodríguez Alén, AgustínRoselló, ElenaTorres Miñana, LauraMarcellini Antonio, ShallyMoretó Quinana, AnaRodríguez García, José AntonioAguinaco Culebras, ReyesAlonso Escobar, NievesCervero Santiago, CarlosFernández Mosteirín, NúriaMartínez Badás, María PazPérez Sánchez, MontserratPérez Montes, RocíoRodríguez González, RamónUribe Barrientos, MarisolCaparrós Miranda, Isabel SocorroIglesias Fernández, MiriamBaena, ÁngelaRodríguez López, ManuelSebrango Sandia, AnaVázquez Fernández, IreneMarco, PascualSpanish Society of Thrombosis and Haemostasis (SETH)HemofíliaEspanyaHemophiliaSpainThe 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.American Society of Hematology2021info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2445/180958Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))reponame:Dipòsit Digital de la UBinstname:Universidad de BarcelonaInglésReproducció del document publicat a: https://doi.org/10.1182/bloodadvances.2021004626Blood Advances, 2021, vol. 5, num. 19, p. 3821-3829https://doi.org/10.1182/bloodadvances.2021004626(c) American Society of Hematology, 2021info:eu-repo/semantics/openAccessoai:diposit.ub.edu:2445/1809582026-05-27T06:46:51Z
dc.title.none.fl_str_mv	Management of acquired hemophilia A: results from the Spanish registry
title	Management of acquired hemophilia A: results from the Spanish registry
spellingShingle	Management of acquired hemophilia A: results from the Spanish registry Mingot Castellano, María Eva Hemofília Espanya Hemophilia Spain
title_short	Management of acquired hemophilia A: results from the Spanish registry
title_full	Management of acquired hemophilia A: results from the Spanish registry
title_fullStr	Management of acquired hemophilia A: results from the Spanish registry
title_full_unstemmed	Management of acquired hemophilia A: results from the Spanish registry
title_sort	Management of acquired hemophilia A: results from the Spanish registry
dc.creator.none.fl_str_mv	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)
author	Mingot Castellano, María Eva
author_facet	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)
author_role	author
author2	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)
author2_role	author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author
dc.subject.none.fl_str_mv	Hemofília Espanya Hemophilia Spain
topic	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.
publishDate	2021
dc.date.none.fl_str_mv	2021
dc.type.none.fl_str_mv	info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion
format	article
status_str	publishedVersion
dc.identifier.none.fl_str_mv	https://hdl.handle.net/2445/180958
url	https://hdl.handle.net/2445/180958
dc.language.none.fl_str_mv	Inglés
language_invalid_str_mv	Inglés
dc.relation.none.fl_str_mv	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
dc.rights.none.fl_str_mv	(c) American Society of Hematology, 2021 info:eu-repo/semantics/openAccess
rights_invalid_str_mv	(c) American Society of Hematology, 2021
eu_rights_str_mv	openAccess
dc.format.none.fl_str_mv	application/pdf
dc.publisher.none.fl_str_mv	American Society of Hematology
publisher.none.fl_str_mv	American Society of Hematology
dc.source.none.fl_str_mv	Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) reponame:Dipòsit Digital de la UB instname:Universidad de Barcelona
instname_str	Universidad de Barcelona
reponame_str	Dipòsit Digital de la UB
collection	Dipòsit Digital de la UB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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Management of acquired hemophilia A: results from the Spanish registry

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