Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry

Background: Limited data exist about the clinical presentation, ideal therapy and outcomes of patients with hereditary hemorrhagic telangiectasia (HHT) who develop venous thromboembolism (VTE). Methods: We used the data in the RIETE Registry to assess the clinical characteristics, therapeutic approa...

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Autores: Riera Mestre, Antoni|||0000-0001-9411-804X, Mora Luján, José María|||0000-0002-5333-2389, Trujillo-Santos, Javier|||0000-0003-0861-9927, Del Toro, Jorge|||0000-0003-1791-9550, Nieto, José Antonio, Pedrajas, José María|||0000-0002-3597-1398, López-Reyes, Raquel, Soler, Sílvia (Soler Simón), Ballaz, Aitor, Cerdà, Pau|||0000-0003-4368-6260, Monreal, Manel
Tipo de recurso: artículo
Fecha de publicación:2019
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:223027
Acceso en línea:https://ddd.uab.cat/record/223027
https://dx.doi.org/urn:doi:10.1186/s13023-019-1172-8
Access Level:acceso abierto
Palabra clave:Deep venous thrombosis
Hemorrhagic hereditary telangiectasia
Pulmonary embolism
Rare diseases
Venous thromboembolism
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spelling Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registryRiera Mestre, Antoni|||0000-0001-9411-804XMora Luján, José María|||0000-0002-5333-2389Trujillo-Santos, Javier|||0000-0003-0861-9927Del Toro, Jorge|||0000-0003-1791-9550Nieto, José AntonioPedrajas, José María|||0000-0002-3597-1398López-Reyes, RaquelSoler, Sílvia (Soler Simón)Ballaz, AitorCerdà, Pau|||0000-0003-4368-6260Monreal, ManelDeep venous thrombosisHemorrhagic hereditary telangiectasiaPulmonary embolismRare diseasesVenous thromboembolismBackground: Limited data exist about the clinical presentation, ideal therapy and outcomes of patients with hereditary hemorrhagic telangiectasia (HHT) who develop venous thromboembolism (VTE). Methods: We used the data in the RIETE Registry to assess the clinical characteristics, therapeutic approaches and clinical outcomes during the course of anticoagulant therapy in patients with HHT according to initial presentation as pulmonary embolism (PE) or deep venous thrombosis (DVT). Results: Of 51,375 patients with acute VTE enrolled in RIETE from February 2009 to January 2019, 23 (0.04%) had HHT: 14 (61%) initially presented with PE and 9 (39%) with DVT alone. Almost half (47.8%) of the patients with VTE had a risk factor for VTE. Most PE and DVT patients received low-molecular-weight heparin for initial (71 and 100%, respectively) and long-term therapy (54 and 67%, respectively). During anticoagulation for VTE, the rate of bleeding events (major 2, non-major 6) far outweighed the rate of VTE recurrences (recurrent DVT 1): 50.1 bleeds per 100 patient-years (95%CI: 21.6-98.7) vs. 6.26 recurrences (95%CI: 0.31-30.9; p = 0.020). One major and three non-major bleeding were epistaxis. No patient died of bleeding. One patient died shortly after being diagnosed with acute PE. Conclusions: During anticoagulation for VTE in HHT patients, there were more bleeding events than VTE recurrences. Most bleeding episodes were non-major epistaxis.Universitat Autònoma de Barcelona 22019-01-0120192019-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/223027https://dx.doi.org/urn:doi:10.1186/s13023-019-1172-8reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:2230272026-06-06T12:50:31Z
dc.title.none.fl_str_mv Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
title Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
spellingShingle Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
Riera Mestre, Antoni|||0000-0001-9411-804X
Deep venous thrombosis
Hemorrhagic hereditary telangiectasia
Pulmonary embolism
Rare diseases
Venous thromboembolism
title_short Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
title_full Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
title_fullStr Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
title_full_unstemmed Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
title_sort Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
dc.creator.none.fl_str_mv Riera Mestre, Antoni|||0000-0001-9411-804X
Mora Luján, José María|||0000-0002-5333-2389
Trujillo-Santos, Javier|||0000-0003-0861-9927
Del Toro, Jorge|||0000-0003-1791-9550
Nieto, José Antonio
Pedrajas, José María|||0000-0002-3597-1398
López-Reyes, Raquel
Soler, Sílvia (Soler Simón)
Ballaz, Aitor
Cerdà, Pau|||0000-0003-4368-6260
Monreal, Manel
author Riera Mestre, Antoni|||0000-0001-9411-804X
author_facet Riera Mestre, Antoni|||0000-0001-9411-804X
Mora Luján, José María|||0000-0002-5333-2389
Trujillo-Santos, Javier|||0000-0003-0861-9927
Del Toro, Jorge|||0000-0003-1791-9550
Nieto, José Antonio
Pedrajas, José María|||0000-0002-3597-1398
López-Reyes, Raquel
Soler, Sílvia (Soler Simón)
Ballaz, Aitor
Cerdà, Pau|||0000-0003-4368-6260
Monreal, Manel
author_role author
author2 Mora Luján, José María|||0000-0002-5333-2389
Trujillo-Santos, Javier|||0000-0003-0861-9927
Del Toro, Jorge|||0000-0003-1791-9550
Nieto, José Antonio
Pedrajas, José María|||0000-0002-3597-1398
López-Reyes, Raquel
Soler, Sílvia (Soler Simón)
Ballaz, Aitor
Cerdà, Pau|||0000-0003-4368-6260
Monreal, Manel
author2_role author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universitat Autònoma de Barcelona
dc.subject.none.fl_str_mv Deep venous thrombosis
Hemorrhagic hereditary telangiectasia
Pulmonary embolism
Rare diseases
Venous thromboembolism
topic Deep venous thrombosis
Hemorrhagic hereditary telangiectasia
Pulmonary embolism
Rare diseases
Venous thromboembolism
description Background: Limited data exist about the clinical presentation, ideal therapy and outcomes of patients with hereditary hemorrhagic telangiectasia (HHT) who develop venous thromboembolism (VTE). Methods: We used the data in the RIETE Registry to assess the clinical characteristics, therapeutic approaches and clinical outcomes during the course of anticoagulant therapy in patients with HHT according to initial presentation as pulmonary embolism (PE) or deep venous thrombosis (DVT). Results: Of 51,375 patients with acute VTE enrolled in RIETE from February 2009 to January 2019, 23 (0.04%) had HHT: 14 (61%) initially presented with PE and 9 (39%) with DVT alone. Almost half (47.8%) of the patients with VTE had a risk factor for VTE. Most PE and DVT patients received low-molecular-weight heparin for initial (71 and 100%, respectively) and long-term therapy (54 and 67%, respectively). During anticoagulation for VTE, the rate of bleeding events (major 2, non-major 6) far outweighed the rate of VTE recurrences (recurrent DVT 1): 50.1 bleeds per 100 patient-years (95%CI: 21.6-98.7) vs. 6.26 recurrences (95%CI: 0.31-30.9; p = 0.020). One major and three non-major bleeding were epistaxis. No patient died of bleeding. One patient died shortly after being diagnosed with acute PE. Conclusions: During anticoagulation for VTE in HHT patients, there were more bleeding events than VTE recurrences. Most bleeding episodes were non-major epistaxis.
publishDate 2019
dc.date.none.fl_str_mv 2
2019-01-01
2019
2019-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/223027
https://dx.doi.org/urn:doi:10.1186/s13023-019-1172-8
url https://ddd.uab.cat/record/223027
https://dx.doi.org/urn:doi:10.1186/s13023-019-1172-8
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
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