Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor

Autores
Kelly, Claire; Thomas, William; Baker, Ross I.; O'Donnell, James S.; Sánchez Luceros, Analía Gabriela; Lavin, Michelle
Año de publicación
2024
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Background: Bleeding disorder of unknown cause (BDUC) is characterised by a bleeding phenotype in the setting of normal haemostatic testing. No standardised diagnostic criteria or treatment algorithms exist for people with BDUC. To address the unmet need, the International Society on Thrombosis and Haemostasis von Willebrand Factor Scientific Subcommittee (ISTH VWF SCC) performed a real-world survey, aimed at addressing knowledge gaps, developing consensus pathways and ultimately improving care. Objectives and Methods: We sought to determine current international clinical practices in the investigation, registration, and treatment of people with BDUC through an online structured survey of health care providers (HCPs) who managed patient with bleeding disorders. Results: Two hundred and sixteen respondents from 39 countries were included in the final analysis. The clinical assessment of those with a possible bleeding disorder varied, with only 55% excluding hypermobility but high levels (80%) of bleeding assessment tools (BAT) usage. In haemostatic testing only the prothrombin time (PT) and activated partial thromboplastin time (APTT) tests gained universal support. Tranexamic acid (TXA) was favoured for prophylaxis for minor (71%)/major (59%) surgeries and pregnancy (58%) but advice on the treatment advised if bleeding occurred was heterogeneous. The management of heavy menstrual bleeding (HMB) in women despite combined oral contraceptive pill (COCP) use also proved challenging with HCPs selecting multiple alternative strategies. Conclusion: Significant variation exists in the recognition, registration and management of people with BDUC worldwide. This survey emphasises the need for consensus pathways to diagnose and treat BDUC to standardise and improve care for patients internationally.
Fil: Kelly, Claire. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda
Fil: Thomas, William. University of Cambridge; Reino Unido
Fil: Baker, Ross I.. Murdoch University; Australia. Hollywood Hospital Haemophilia Treatment Centre; Australia. Irish-Australian Blood Collaborative Network; Irlanda
Fil: O'Donnell, James S.. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda. Irish-Australian Blood Collaborative Network; Irlanda
Fil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex". Departamento de Hemostasia y Trombosis; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Lavin, Michelle. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda
Materia
BLEEDING DISORDERS OF UNKNOWN CAUSE
INHERITED COAGULATION DISORDERS
HAEMOSTASIS
PREGNANCY
SURGERY
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/265090
Acceder
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oai_identifier_str oai:ri.conicet.gov.ar:11336/265090
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand FactorKelly, ClaireThomas, WilliamBaker, Ross I.O'Donnell, James S.Sánchez Luceros, Analía GabrielaLavin, MichelleBLEEDING DISORDERS OF UNKNOWN CAUSEINHERITED COAGULATION DISORDERSHAEMOSTASISPREGNANCYSURGERYhttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Background: Bleeding disorder of unknown cause (BDUC) is characterised by a bleeding phenotype in the setting of normal haemostatic testing. No standardised diagnostic criteria or treatment algorithms exist for people with BDUC. To address the unmet need, the International Society on Thrombosis and Haemostasis von Willebrand Factor Scientific Subcommittee (ISTH VWF SCC) performed a real-world survey, aimed at addressing knowledge gaps, developing consensus pathways and ultimately improving care. Objectives and Methods: We sought to determine current international clinical practices in the investigation, registration, and treatment of people with BDUC through an online structured survey of health care providers (HCPs) who managed patient with bleeding disorders. Results: Two hundred and sixteen respondents from 39 countries were included in the final analysis. The clinical assessment of those with a possible bleeding disorder varied, with only 55% excluding hypermobility but high levels (80%) of bleeding assessment tools (BAT) usage. In haemostatic testing only the prothrombin time (PT) and activated partial thromboplastin time (APTT) tests gained universal support. Tranexamic acid (TXA) was favoured for prophylaxis for minor (71%)/major (59%) surgeries and pregnancy (58%) but advice on the treatment advised if bleeding occurred was heterogeneous. The management of heavy menstrual bleeding (HMB) in women despite combined oral contraceptive pill (COCP) use also proved challenging with HCPs selecting multiple alternative strategies. Conclusion: Significant variation exists in the recognition, registration and management of people with BDUC worldwide. This survey emphasises the need for consensus pathways to diagnose and treat BDUC to standardise and improve care for patients internationally.Fil: Kelly, Claire. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; IrlandaFil: Thomas, William. University of Cambridge; Reino UnidoFil: Baker, Ross I.. Murdoch University; Australia. Hollywood Hospital Haemophilia Treatment Centre; Australia. Irish-Australian Blood Collaborative Network; IrlandaFil: O'Donnell, James S.. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda. Irish-Australian Blood Collaborative Network; IrlandaFil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex". Departamento de Hemostasia y Trombosis; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaFil: Lavin, Michelle. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; IrlandaElsevier2024-06info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/265090Kelly, Claire; Thomas, William; Baker, Ross I.; O'Donnell, James S.; Sánchez Luceros, Analía Gabriela; et al.; Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor; Elsevier; Journal of Thrombosis and Haemostasis; 22; 10; 6-2024; 2900-29091538-7836CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S1538783624003192info:eu-repo/semantics/altIdentifier/doi/10.1016/j.jtha.2024.05.027info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-nd/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-29T10:02:37Zoai:ri.conicet.gov.ar:11336/265090instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-29 10:02:37.656CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
title Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
spellingShingle Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
Kelly, Claire
BLEEDING DISORDERS OF UNKNOWN CAUSE
INHERITED COAGULATION DISORDERS
HAEMOSTASIS
PREGNANCY
SURGERY
title_short Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
title_full Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
title_fullStr Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
title_full_unstemmed Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
title_sort Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor
dc.creator.none.fl_str_mv Kelly, Claire
Thomas, William
Baker, Ross I.
O'Donnell, James S.
Sánchez Luceros, Analía Gabriela
Lavin, Michelle
author Kelly, Claire
author_facet Kelly, Claire
Thomas, William
Baker, Ross I.
O'Donnell, James S.
Sánchez Luceros, Analía Gabriela
Lavin, Michelle
author_role author
author2 Thomas, William
Baker, Ross I.
O'Donnell, James S.
Sánchez Luceros, Analía Gabriela
Lavin, Michelle
author2_role author
author
author
author
author
dc.subject.none.fl_str_mv BLEEDING DISORDERS OF UNKNOWN CAUSE
INHERITED COAGULATION DISORDERS
HAEMOSTASIS
PREGNANCY
SURGERY
topic BLEEDING DISORDERS OF UNKNOWN CAUSE
INHERITED COAGULATION DISORDERS
HAEMOSTASIS
PREGNANCY
SURGERY
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Background: Bleeding disorder of unknown cause (BDUC) is characterised by a bleeding phenotype in the setting of normal haemostatic testing. No standardised diagnostic criteria or treatment algorithms exist for people with BDUC. To address the unmet need, the International Society on Thrombosis and Haemostasis von Willebrand Factor Scientific Subcommittee (ISTH VWF SCC) performed a real-world survey, aimed at addressing knowledge gaps, developing consensus pathways and ultimately improving care. Objectives and Methods: We sought to determine current international clinical practices in the investigation, registration, and treatment of people with BDUC through an online structured survey of health care providers (HCPs) who managed patient with bleeding disorders. Results: Two hundred and sixteen respondents from 39 countries were included in the final analysis. The clinical assessment of those with a possible bleeding disorder varied, with only 55% excluding hypermobility but high levels (80%) of bleeding assessment tools (BAT) usage. In haemostatic testing only the prothrombin time (PT) and activated partial thromboplastin time (APTT) tests gained universal support. Tranexamic acid (TXA) was favoured for prophylaxis for minor (71%)/major (59%) surgeries and pregnancy (58%) but advice on the treatment advised if bleeding occurred was heterogeneous. The management of heavy menstrual bleeding (HMB) in women despite combined oral contraceptive pill (COCP) use also proved challenging with HCPs selecting multiple alternative strategies. Conclusion: Significant variation exists in the recognition, registration and management of people with BDUC worldwide. This survey emphasises the need for consensus pathways to diagnose and treat BDUC to standardise and improve care for patients internationally.
Fil: Kelly, Claire. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda
Fil: Thomas, William. University of Cambridge; Reino Unido
Fil: Baker, Ross I.. Murdoch University; Australia. Hollywood Hospital Haemophilia Treatment Centre; Australia. Irish-Australian Blood Collaborative Network; Irlanda
Fil: O'Donnell, James S.. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda. Irish-Australian Blood Collaborative Network; Irlanda
Fil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex". Departamento de Hemostasia y Trombosis; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Lavin, Michelle. St James's Hospital; Irlanda. Irish Centre For Vascular Biology; Irlanda
description Background: Bleeding disorder of unknown cause (BDUC) is characterised by a bleeding phenotype in the setting of normal haemostatic testing. No standardised diagnostic criteria or treatment algorithms exist for people with BDUC. To address the unmet need, the International Society on Thrombosis and Haemostasis von Willebrand Factor Scientific Subcommittee (ISTH VWF SCC) performed a real-world survey, aimed at addressing knowledge gaps, developing consensus pathways and ultimately improving care. Objectives and Methods: We sought to determine current international clinical practices in the investigation, registration, and treatment of people with BDUC through an online structured survey of health care providers (HCPs) who managed patient with bleeding disorders. Results: Two hundred and sixteen respondents from 39 countries were included in the final analysis. The clinical assessment of those with a possible bleeding disorder varied, with only 55% excluding hypermobility but high levels (80%) of bleeding assessment tools (BAT) usage. In haemostatic testing only the prothrombin time (PT) and activated partial thromboplastin time (APTT) tests gained universal support. Tranexamic acid (TXA) was favoured for prophylaxis for minor (71%)/major (59%) surgeries and pregnancy (58%) but advice on the treatment advised if bleeding occurred was heterogeneous. The management of heavy menstrual bleeding (HMB) in women despite combined oral contraceptive pill (COCP) use also proved challenging with HCPs selecting multiple alternative strategies. Conclusion: Significant variation exists in the recognition, registration and management of people with BDUC worldwide. This survey emphasises the need for consensus pathways to diagnose and treat BDUC to standardise and improve care for patients internationally.
publishDate 2024
dc.date.none.fl_str_mv 2024-06
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/265090
Kelly, Claire; Thomas, William; Baker, Ross I.; O'Donnell, James S.; Sánchez Luceros, Analía Gabriela; et al.; Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor; Elsevier; Journal of Thrombosis and Haemostasis; 22; 10; 6-2024; 2900-2909
1538-7836
CONICET Digital
CONICET
url http://hdl.handle.net/11336/265090
identifier_str_mv Kelly, Claire; Thomas, William; Baker, Ross I.; O'Donnell, James S.; Sánchez Luceros, Analía Gabriela; et al.; Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor; Elsevier; Journal of Thrombosis and Haemostasis; 22; 10; 6-2024; 2900-2909
1538-7836
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S1538783624003192
info:eu-repo/semantics/altIdentifier/doi/10.1016/j.jtha.2024.05.027
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.070432

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