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Orphanet J Rare Dis. 2019 Aug 9;14(1):196. doi: 10.1186/s13023-019-1172-8.

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

Collaborators (193)

Monreal M, Prandoni P, Brenner B, Farge-Bancel D, Barba R, Di Micco P, Bertoletti L, Schellong S, Tzoran I, Reis A, Bosevski M, Bounameaux H, Malý R, Verhamme P, Caprini JA, Bui HM, Adarraga MD, Agud M, Aibar MA, Alcalde-Manero M, Alfonso J, Amado C, Arcelus JI, Ballaz A, Barba R, Barbagelata C, Barrón M, Barrón-Andrés B, Blanco-Molina A, Camon AM, Cañas I, Castro J, Cerdà P, de Miguel J, Del Toro J, Demelo P, Díaz-Pedroche C, Díaz-Peromingo JA, Domínguez IM, Escribano JC, Falgá C, Fernández-Capitán C, Fernández-Criado MC, Fidalgo MA, Flores K, Font C, Font L, Furest I, García MA, García-Bragado F, García-Raso A, Gavín-Blanco O, Gavín-Sebastián O, Gil-Díaz A, Godoy-Díaz D, Gómez V, Gómez-Cuervo C, González-Martínez J, Grau E, Guirado L, Gutiérrez J, Hernández-Blasco LM, Jara-Palomares L, Jaras MJ, Jiménez D, Joya MD, Jou I, Lalueza A, Lecumberri R, Lima J, Llamas P, Lobo JL, López-Jiménez L, López-Meseguer M, López-Miguel P, López-Núñez JJ, López-Reyes R, López-Sáez JB, Lorente MA, Loring M, Lumbierres M, Madridano O, Maestre A, Marchena PJ, Martín-Martos F, Martínez-Baquerizo C, Martínez-García MA, Mellado M, Moisés J, Monreal M, Morales MV, Muñoz-Blanco A, Nieto JA, Núñez MJ, Olivares MC, Olivera PE, Ortega C, Osorio J, Otalora S, Otero R, Panadero-Macia M, Parra V, Pedrajas JM, Pellejero G, Pérez-Ductor C, Pérez-Rus G, Peris ML, Pesantez D, Porras JA, Riera-Mestre A, Rivas A, Rodríguez-Cobo A, Rodríguez-Matute C, Rosa V, Rubio CM, Ruiz-Artacho P, Ruiz-Sada P, Sahuquillo JC, Sala-Sainz MC, Salgueiro G, Sampériz A, Sánchez-Martínez R, Sánchez-Muñoz-Torrero JF, Seguí E, Soler S, Suárez S, Suriñach JM, Tolosa C, Torres MI, Trujillo-Santos J, Uresandi F, Valero B, Valle R, Vidal G, Vilar C, Villares P, Gutiérrez P, Vázquez FJ, Vilaseca A, Vanassche T, Vandenbriele C, Verhamme P, Hirmerova J, Malý R, Salgado E, Benzidia I, Bertoletti L, Bura-Riviere A, Debourdeau P, Courtois MC, Farge-Bancel D, Helfer H, Hij A, Mahé I, Moustafa F, Schellong S, Braester A, Brenner B, Tzoran I, Bilora F, Bortoluzzi C, Ciammaichella M, Dentali F, Di Micco P, Ferrazzi P, Imbalzano E, Lodigiani C, Maida R, Mastroiacovo D, Mumoli N, Pace F, Pesavento R, Pomero F, Prandoni P, Quintavalla R, Rocci A, Rota L, Siniscalchi C, Tiraferri E, Tufano A, Visonà A, Vo Hong N, Zalunardo B, Kalejs RV, Kigitovica D, Skride A, Bosevski M, Zdraveska M, Bounameaux H, Mazzolai L, Caprini JA, Tafur AJ, Bui HM.

Author information

1
HHT Unit and VTE Unit, Internal Medicine Department, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), C/ Feixa Llarga s/n. L'Hospitalet de Llobregat, 08907, Barcelona, Spain. ariera@bellvitgehospital.cat.
2
Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain. ariera@bellvitgehospital.cat.
3
HHT Unit and VTE Unit, Internal Medicine Department, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), C/ Feixa Llarga s/n. L'Hospitalet de Llobregat, 08907, Barcelona, Spain.
4
Department of Internal Medicine, Hospital General Universitario Santa Lucía, Cartagena, Universidad Católica de Murcia, Murcia, Spain.
5
Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
6
Department of Internal Medicine, Hospital General Virgen De La Luz, Cuenca, Spain.
7
Department of Internal Medicine, Hospital Clínico San Carlos, Madrid, Spain.
8
Department of Pneumonology, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
9
Department of Internal Medicine, Fundació Hospital d'Olot i Comarcal de la Garrotxa, Gerona, Spain.
10
Department of Pneumonology, Hospital De Galdakao, Galdakao, Vizcaya, Spain.
11
Department of Internal Medicine, Hospital Germans Trias i Pujol Badalona, Barcelona, Universidad Autónoma de Barcelona, Barcelona, Spain.

Abstract

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.

KEYWORDS:

Deep venous thrombosis; Hemorrhagic hereditary telangiectasia; Pulmonary embolism; Rare diseases; Venous thromboembolism

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