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

Antoni Riera-Mestre; José María Mora-Luján; Javier Trujillo-Santos; Jorge Del Toro; José Antonio Nieto; José María Pedrajas; Raquel López-Reyes; Silvia Soler; Aitor Ballaz; Pau Cerdà; Manel Monreal; Paolo Prandoni; Benjamin Brenner; Dominique Farge-Bancel; Raquel Barba; Pierpaolo Di Micco; Laurent Bertoletti; Sebastian Schellong; Inna Tzoran; Abilio Reis; Marijan Bosevski; Henri Bounameaux; Radovan Malý; Peter Verhamme; Joseph A. Caprini; Hanh My Bui; M. D. Adarraga; M. Agud; M. A. Aibar; M. Alcalde-Manero; J. Alfonso; C. Amado; J. I. Arcelus; A. Ballaz; R. Barba; C. Barbagelata; M. Barrón; B. Barrón-Andrés; A. Blanco-Molina; A. M. Camon; I. Cañas; J. Castro; P. Cerdà; J. De Miguel; Jorge Del Toro; P. Demelo; C. Díaz-Pedroche; J. A. Díaz-Peromingo; I. M. Domínguez; J. C. Escribano; C. Falgá; C. Fernández-Capitán; M. C. Fernández-Criado; M. A. Fidalgo; K. Flores; C. Font; L. Font; I. Furest; M. A. García; F. García-Bragado; A. García-Raso; O. Gavín-Blanco; O. Gavín-Sebastián; A. Gil-Díaz; D. Godoy-Díaz; V. Gómez; C. Gómez-Cuervo; J. González-Martínez; E. Grau; L. Guirado; J. Gutiérrez; L. M. Hernández-Blasco; L. Jara-Palomares; M. J. Jaras; D. Jiménez; M. D. Joya; I. Jou; A. Lalueza; R. Lecumberri; J. Lima; P. Llamas; J. L. Lobo; L. López-Jiménez; M. López-Meseguer; P. López-Miguel; J. J. López-Núñez; R. López-Reyes; J. B. López-Sáez; M. A. Lorente; M. Loring; M. Lumbierres; O. Madridano; A. Maestre; P. J. Marchena; F. Martín-Martos; C. Martínez-Baquerizo; M. A. Martínez-García; J. Moisés

doi:10.1186/s13023-019-1172-8

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

Antoni Riera-Mestre, José María Mora-Luján, Javier Trujillo-Santos, Jorge Del Toro, José Antonio Nieto, José María Pedrajas, Raquel López-Reyes, Silvia Soler, Aitor Ballaz, Pau Cerdà, Manel Monreal, Paolo Prandoni, Benjamin Brenner, Dominique Farge-Bancel, Raquel Barba, Pierpaolo Di Micco, Laurent Bertoletti, Sebastian Schellong, Inna Tzoran, Abilio ReisMarijan Bosevski, Henri Bounameaux, Radovan Malý, Peter Verhamme, Joseph A. Caprini, Hanh My Bui, M. D. Adarraga, M. Agud, M. A. Aibar, M. Alcalde-Manero, J. Alfonso, C. Amado, J. I. Arcelus, A. Ballaz, R. Barba, C. Barbagelata, M. Barrón, B. Barrón-Andrés, A. Blanco-Molina, A. M. Camon, I. Cañas, J. Castro, P. Cerdà, J. De Miguel, Jorge Del Toro, P. Demelo, C. Díaz-Pedroche, J. A. Díaz-Peromingo, I. M. Domínguez, J. C. Escribano, C. Falgá, C. Fernández-Capitán, M. C. Fernández-Criado, M. A. Fidalgo, K. Flores, C. Font, L. Font, I. Furest, M. A. García, F. García-Bragado, A. García-Raso, O. Gavín-Blanco, O. Gavín-Sebastián, A. Gil-Díaz, D. Godoy-Díaz, V. Gómez, C. Gómez-Cuervo, J. González-Martínez, E. Grau, L. Guirado, J. Gutiérrez, L. M. Hernández-Blasco, L. Jara-Palomares, M. J. Jaras, D. Jiménez, M. D. Joya, I. Jou, A. Lalueza, R. Lecumberri, J. Lima, P. Llamas, J. L. Lobo, L. López-Jiménez, M. López-Meseguer, P. López-Miguel, J. J. López-Núñez, R. López-Reyes, J. B. López-Sáez, M. A. Lorente, M. Loring, M. Lumbierres, O. Madridano, A. Maestre, P. J. Marchena, F. Martín-Martos, C. Martínez-Baquerizo, M. A. Martínez-García, J. Moisés

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Abstract

© 2019 The Author(s). 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.

Original language	English
Article number	196
Pages (from-to)	196
Number of pages	8
Journal	Orphanet Journal of Rare Diseases
Volume	14
Issue number	1
DOIs	https://doi.org/10.1186/s13023-019-1172-8
Publication status	Published - 9 Aug 2019

Keywords

Deep venous thrombosis
EPISTAXIS
FACTOR-VIII
HHT
Hemorrhagic hereditary telangiectasia
OUTCOMES
Pulmonary embolism
RENDU-OSLER-WEBER
Rare diseases
THERAPY
Venous thromboembolism

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10.1186/s13023-019-1172-8

https://ddd.uab.cat/record/223027

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Riera-Mestre, A., Mora-Luján, J. M., Trujillo-Santos, J., Del Toro, J., Nieto, J. A., Pedrajas, J. M., López-Reyes, R., Soler, S., Ballaz, A., Cerdà, P., Monreal, M., Prandoni, P., Brenner, B., Farge-Bancel, D., Barba, R., Di Micco, P., Bertoletti, L., Schellong, S., Tzoran, I., ... Moisés, J. (2019). Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry. Orphanet Journal of Rare Diseases, 14(1), 196. Article 196. https://doi.org/10.1186/s13023-019-1172-8

@article{9c659af56b5e4032a479df2967018e9d,

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

abstract = "{\textcopyright} 2019 The Author(s). 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, EPISTAXIS, FACTOR-VIII, HHT, Hemorrhagic hereditary telangiectasia, OUTCOMES, Pulmonary embolism, RENDU-OSLER-WEBER, Rare diseases, THERAPY, Venous thromboembolism",

author = "Antoni Riera-Mestre and Mora-Luj{\'a}n, {Jos{\'e} Mar{\'i}a} and Javier Trujillo-Santos and {Del Toro}, Jorge and Nieto, {Jos{\'e} Antonio} and Pedrajas, {Jos{\'e} Mar{\'i}a} and Raquel L{\'o}pez-Reyes and Silvia Soler and Aitor Ballaz and Pau Cerd{\`a} and Manel Monreal and Paolo Prandoni and Benjamin Brenner and Dominique Farge-Bancel and Raquel Barba and {Di Micco}, Pierpaolo and Laurent Bertoletti and Sebastian Schellong and Inna Tzoran and Abilio Reis and Marijan Bosevski and Henri Bounameaux and Radovan Mal{\'y} and Peter Verhamme and Caprini, {Joseph A.} and Bui, {Hanh My} and Adarraga, {M. D.} and M. Agud and Aibar, {M. A.} and M. Alcalde-Manero and J. Alfonso and C. Amado and Arcelus, {J. I.} and A. Ballaz and R. Barba and C. Barbagelata and M. Barr{\'o}n and B. Barr{\'o}n-Andr{\'e}s and A. Blanco-Molina and Camon, {A. M.} and I. Ca{\~n}as and J. Castro and P. Cerd{\`a} and {De Miguel}, J. and {Del Toro}, Jorge and P. Demelo and C. D{\'i}az-Pedroche and D{\'i}az-Peromingo, {J. A.} and Dom{\'i}nguez, {I. M.} and Escribano, {J. C.} and C. Falg{\'a} and C. Fern{\'a}ndez-Capit{\'a}n and Fern{\'a}ndez-Criado, {M. C.} and Fidalgo, {M. A.} and K. Flores and C. Font and L. Font and I. Furest and Garc{\'i}a, {M. A.} and F. Garc{\'i}a-Bragado and A. Garc{\'i}a-Raso and O. Gav{\'i}n-Blanco and O. Gav{\'i}n-Sebasti{\'a}n and A. Gil-D{\'i}az and D. Godoy-D{\'i}az and V. G{\'o}mez and C. G{\'o}mez-Cuervo and J. Gonz{\'a}lez-Mart{\'i}nez and E. Grau and L. Guirado and J. Guti{\'e}rrez and Hern{\'a}ndez-Blasco, {L. M.} and L. Jara-Palomares and Jaras, {M. J.} and D. Jim{\'e}nez and Joya, {M. D.} and I. Jou and A. Lalueza and R. Lecumberri and J. Lima and P. Llamas and Lobo, {J. L.} and L. L{\'o}pez-Jim{\'e}nez and M. L{\'o}pez-Meseguer and P. L{\'o}pez-Miguel and L{\'o}pez-N{\'u}{\~n}ez, {J. J.} and R. L{\'o}pez-Reyes and L{\'o}pez-S{\'a}ez, {J. B.} and Lorente, {M. A.} and M. Loring and M. Lumbierres and O. Madridano and A. Maestre and Marchena, {P. J.} and F. Mart{\'i}n-Martos and C. Mart{\'i}nez-Baquerizo and Mart{\'i}nez-Garc{\'i}a, {M. A.} and J. Mois{\'e}s",

year = "2019",

month = aug,

day = "9",

doi = "10.1186/s13023-019-1172-8",

language = "English",

volume = "14",

pages = "196",

journal = "Orphanet Journal of Rare Diseases",

issn = "1750-1172",

number = "1",

}

Riera-Mestre, A, Mora-Luján, JM, Trujillo-Santos, J, Del Toro, J, Nieto, JA, Pedrajas, JM, López-Reyes, R, Soler, S, Ballaz, A, Cerdà, P, 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 & Moisés, J 2019, 'Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry', Orphanet Journal of Rare Diseases, vol. 14, no. 1, 196, pp. 196. https://doi.org/10.1186/s13023-019-1172-8

TY - JOUR

T1 - Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry

AU - Riera-Mestre, Antoni

AU - Mora-Luján, José María

AU - Trujillo-Santos, Javier

AU - Del Toro, Jorge

AU - Nieto, José Antonio

AU - Pedrajas, José María

AU - López-Reyes, Raquel

AU - Soler, Silvia

AU - Ballaz, Aitor

AU - Cerdà, Pau

AU - Monreal, Manel

AU - Prandoni, Paolo

AU - Brenner, Benjamin

AU - Farge-Bancel, Dominique

AU - Barba, Raquel

AU - Di Micco, Pierpaolo

AU - Bertoletti, Laurent

AU - Schellong, Sebastian

AU - Tzoran, Inna

AU - Reis, Abilio

AU - Bosevski, Marijan

AU - Bounameaux, Henri

AU - Malý, Radovan

AU - Verhamme, Peter

AU - Caprini, Joseph A.

AU - Bui, Hanh My

AU - Adarraga, M. D.

AU - Agud, M.

AU - Aibar, M. A.

AU - Alcalde-Manero, M.

AU - Alfonso, J.

AU - Amado, C.

AU - Arcelus, J. I.

AU - Ballaz, A.

AU - Barba, R.

AU - Barbagelata, C.

AU - Barrón, M.

AU - Barrón-Andrés, B.

AU - Blanco-Molina, A.

AU - Camon, A. M.

AU - Cañas, I.

AU - Castro, J.

AU - Cerdà, P.

AU - De Miguel, J.

AU - Del Toro, Jorge

AU - Demelo, P.

AU - Díaz-Pedroche, C.

AU - Díaz-Peromingo, J. A.

AU - Domínguez, I. M.

AU - Escribano, J. C.

AU - Falgá, C.

AU - Fernández-Capitán, C.

AU - Fernández-Criado, M. C.

AU - Fidalgo, M. A.

AU - Flores, K.

AU - Font, C.

AU - Font, L.

AU - Furest, I.

AU - García, M. A.

AU - García-Bragado, F.

AU - García-Raso, A.

AU - Gavín-Blanco, O.

AU - Gavín-Sebastián, O.

AU - Gil-Díaz, A.

AU - Godoy-Díaz, D.

AU - Gómez, V.

AU - Gómez-Cuervo, C.

AU - González-Martínez, J.

AU - Grau, E.

AU - Guirado, L.

AU - Gutiérrez, J.

AU - Hernández-Blasco, L. M.

AU - Jara-Palomares, L.

AU - Jaras, M. J.

AU - Jiménez, D.

AU - Joya, M. D.

AU - Jou, I.

AU - Lalueza, A.

AU - Lecumberri, R.

AU - Lima, J.

AU - Llamas, P.

AU - Lobo, J. L.

AU - López-Jiménez, L.

AU - López-Meseguer, M.

AU - López-Miguel, P.

AU - López-Núñez, J. J.

AU - López-Reyes, R.

AU - López-Sáez, J. B.

AU - Lorente, M. A.

AU - Loring, M.

AU - Lumbierres, M.

AU - Madridano, O.

AU - Maestre, A.

AU - Marchena, P. J.

AU - Martín-Martos, F.

AU - Martínez-Baquerizo, C.

AU - Martínez-García, M. A.

AU - Moisés, J.

PY - 2019/8/9

Y1 - 2019/8/9

N2 - © 2019 The Author(s). 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.

AB - © 2019 The Author(s). 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.

KW - Deep venous thrombosis

KW - EPISTAXIS

KW - FACTOR-VIII

KW - HHT

KW - Hemorrhagic hereditary telangiectasia

KW - OUTCOMES

KW - Pulmonary embolism

KW - RENDU-OSLER-WEBER

KW - Rare diseases

KW - THERAPY

KW - Venous thromboembolism

UR - http://www.mendeley.com/research/natural-history-patients-venous-thromboembolism-hereditary-hemorrhagic-telangiectasia-findings-riete

U2 - 10.1186/s13023-019-1172-8

DO - 10.1186/s13023-019-1172-8

M3 - Article

C2 - 31399146

SN - 1750-1172

VL - 14

SP - 196

JO - Orphanet Journal of Rare Diseases

JF - Orphanet Journal of Rare Diseases

IS - 1

M1 - 196

ER -

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

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