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Hereditary hemorrhagic telangiectasia patients can tolerate anticoagulation

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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by arteriovenous malformations (AVMs) and hemorrhage. HHT patients can also suffer from unrelated medical conditions requiring anticoagulant or antiplatelet treatment (collectively “AT”), though clinicians generally consider HHT a contraindication to AT. We hypothesized that HHT patients can tolerate AT with minimal hemorrhage. Through a chart review of 469 definite HHT patients (1997–2009), we found that 64 (14 %) had received AT. Forty-three out of 64 (67 %) underwent a telephone survey to retrospectively inquire about prescription, early cessation, and HHT-related bleeding. At the time of the study, nine patients were deceased, nine declined, and three were unreachable. During AT treatment, ten (23 %) reported severe complications (blood transfusion, emergency room visit, or hospital admission), while 25 (58 %) reported minor complications (increase or development of epistaxis, gastrointestinal bleeding, and anemia). No patients reported pulmonary/cerebral hemorrhage. The 43 patients received a total of 69 courses of AT. Fourteen out of 69 (20 %) AT courses in 13 patients required early cessation, mostly due to epistaxis. Two out of nine (22 %) deceased patients required early cessation. We conclude that HHT patients can be treated with AT but should be monitored closely given their risk for worsening chronic bleeding and should be screened/treated for pulmonary or cerebral AVMs prior to AT treatment.

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  1. Guttmacher AE, Marchuk DA, White RI Jr (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med 333(14):918–924

    Article  PubMed  CAS  Google Scholar 

  2. Shovlin CL, Sulaiman NL, Govani FS, Jackson JE, Begbie ME (2007) Elevated factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism. Thromb Haemost 98(5):1031–1039. doi:07111031[pii]

    PubMed  CAS  Google Scholar 

  3. Kjeldsen AD, Vase P, Green A (1999) Hereditary haemorrhagic telangiectasia: a population-based study of prevalence and mortality in Danish patients. J Intern Med 245(1):31–39

    Article  PubMed  CAS  Google Scholar 

  4. Lesca G, Olivieri C, Burnichon N, Pagella F, Carette MF, Gilbert-Dussardier B, Goizet C, Roume J, Rabilloud M, Saurin JC, Cottin V, Honnorat J, Coulet F, Giraud S, Calender A, Danesino C, Buscarini E, Plauchu H (2007) Genotype–phenotype correlations in hereditary hemorrhagic telangiectasia: data from the French–Italian HHT network. Genet Med 9(1):14–22

    Article  PubMed  Google Scholar 

  5. Letteboer TG, Mager HJ, Snijder RJ, Lindhout D, Ploos van Amstel HK, Zanen P, Westermann KJ (2008) Genotype–phenotype relationship for localization and age distribution of telangiectases in hereditary hemorrhagic telangiectasia. Am J Med Genet A 146A(21):2733–2739. doi:10.1002/ajmg.a.32243

    Article  PubMed  Google Scholar 

  6. Letteboer TG, Mager JJ, Snijder RJ, Koeleman BP, Lindhout D, Ploos van Amstel JK, Westermann CJ (2006) Genotype–phenotype relationship in hereditary haemorrhagic telangiectasia. J Med Genet 43(4):371–377. doi:10.1136/jmg.2005.035451

    Article  PubMed  CAS  Google Scholar 

  7. Sabba C, Pasculli G, Lenato GM, Suppressa P, Lastella P, Memeo M, Dicuonzo F, Guant G (2007) Hereditary hemorrhagic telangiectasia: clinical features in ENG and ALK1 mutation carriers. J Thromb Haemost 5(6):1149–1157. doi:10.1111/j.1538-7836.2007.02531.x

    Article  PubMed  CAS  Google Scholar 

  8. van Gent MW, Post MC, Luermans JG, Snijder RJ, Westermann CJ, Plokker HW, Overtoom TT, Mager JJ (2008) Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study. Eur Respir J. doi:10.1183/09031936.00049008

  9. Westermann CJ, Rosina AF, De Vries V, de Coteau PA (2003) The prevalence and manifestations of hereditary hemorrhagic telangiectasia in the Afro-Caribbean population of the Netherlands Antilles: a family screening. Am J Med Genet A 116A(4):324–328. doi:10.1002/ajmg.a.10002

    Article  PubMed  Google Scholar 

  10. Plauchu H, de Chadarevian JP, Bideau A, Robert JM (1989) Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 32(3):291–297. doi:10.1002/ajmg.1320320302

    Article  PubMed  CAS  Google Scholar 

  11. Porteous ME, Burn J, Proctor SJ (1992) Hereditary haemorrhagic telangiectasia: a clinical analysis. J Med Genet 29(8):527–530

    Article  PubMed  CAS  Google Scholar 

  12. Assar OS, Friedman CM, White RI Jr (1991) The natural history of epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope 101(9):977–980

    Article  Google Scholar 

  13. Haitjema T, Balder W, Disch FJ, Westermann CJ (1996) Epistaxis in hereditary haemorrhagic telangiectasia. Rhinology 34(3):176–178

    PubMed  CAS  Google Scholar 

  14. Pasculli G, Resta F, Guastamacchia E, Di Gennaro L, Suppressa P, Sabba C (2004) Health-related quality of life in a rare disease: hereditary hemorrhagic telangiectasia (HHT) or Rendu–Osler–Weber disease. Qual Life Res 13(10):1715–1723

    Article  PubMed  Google Scholar 

  15. Geisthoff UW, Heckmann K, D'Amelio R, Grunewald S, Knobber D, Falkai P, Konig J (2007) Health-related quality of life in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 136(5):726–733. doi:10.1016/j.otohns.2006.12.019, discussion 734-725

    Article  PubMed  Google Scholar 

  16. Fulbright RK, Chaloupka JC, Putman CM, Sze GK, Merriam MM, Lee GK, Fayad PB, Awad IA, White RI Jr (1998) MR of hereditary hemorrhagic telangiectasia: prevalence and spectrum of cerebrovascular malformations. AJNR Am J Neuroradiol 19(3):477–484

    PubMed  CAS  Google Scholar 

  17. Haitjema T, Disch F, Overtoom TT, Westermann CJ, Lammers JW (1995) Screening family members of patients with hereditary hemorrhagic telangiectasia. Am J Med 99(5):519–524

    Article  PubMed  CAS  Google Scholar 

  18. Maher CO, Piepgras DG, Brown RD Jr, Friedman JA, Pollock BE (2001) Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. Stroke 32(4):877–882

    Article  PubMed  CAS  Google Scholar 

  19. Ni Bhuachalla CF, O'Connor TM, Murphy M, Colwell N, Brady A (2010) Experience of the Irish National Centre for hereditary haemorrhagic telangiectasia 2003–2008. Respir Med 104(8):1218–1224. doi:10.1016/j.rmed.2010.03.003

    Article  PubMed  CAS  Google Scholar 

  20. Haitjema TJ, Overtoom TT, Westermann CJ, Lammers JW (1995) Embolisation of pulmonary arteriovenous malformations: results and follow up in 32 patients. Thorax 50(7):719–723

    Article  PubMed  CAS  Google Scholar 

  21. Cottin V, Plauchu H, Bayle JY, Barthelet M, Revel D, Cordier JF (2004) Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 169(9):994–1000. doi:10.1164/rccm.200310-1441OC

    Article  PubMed  Google Scholar 

  22. Kjeldsen AD, Kjeldsen J (2000) Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia. Am J Gastroenterol 95(2):415–418. doi:10.1111/j.1572-0241.2000.01792.x

    Article  PubMed  CAS  Google Scholar 

  23. Bayrak-Toydemir P, McDonald J, Markewitz B, Lewin S, Miller F, Chou LS, Gedge F, Tang W, Coon H, Mao R (2006) Genotype–phenotype correlation in hereditary hemorrhagic telangiectasia: mutations and manifestations. Am J Med Genet A 140(5):463–470. doi:10.1002/ajmg.a.31101

    PubMed  Google Scholar 

  24. Sturm W, Gaenzer H, Heininger-Rothbucher D, Neumayr G, Kirchmair R, Patsch J, Weiss G (2003) Unsuspected mesenteric vein thrombosis in a patient with a hereditary bleeding disorder. Blood Coagul Fibrinolysis 14:599–600

    Article  PubMed  Google Scholar 

  25. Goodenberger D, Chakinala M, Picus D (2009) Management of venous thromboembolism in hereditary hemorrhagic telangiectasia. Hematol Meet Rep 3(4):11

    Google Scholar 

  26. Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, Kjeldsen AD, Plauchu H (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber syndrome). Am J Med Genet 91(1):66–67. doi:10.1002/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P

    Article  PubMed  CAS  Google Scholar 

  27. Gallitelli M, Pasculli G, Fiore T, Carella A, Sabba C (2006) Emergencies in hereditary haemorrhagic telangiectasia. QJM: Int J Med 99(1):15–22. doi:10.1093/qjmed/hci148

    Article  CAS  Google Scholar 

  28. Fihn SD, Callahan CM, Martin DC, McDonell MB, Henikoff JG, White RH (1996) The risk for and severity of bleeding complications in elderly patients treated with warfarin. The National Consortium of Anticoagulation Clinics. Ann Intern Med 124(11):970–979

    PubMed  CAS  Google Scholar 

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This study was funded by the Nelson Arthur Hyland Foundation and Li Ka Shing Knowledge Institute (M.E.F.). The authors would like to acknowledge Elaine Granatstein for her assistance in the project.

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The authors declare that they have no conflict of interest.

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Correspondence to Marie E. Faughnan.



Study Name: A Retrospective Study of Outcomes of Anticoagulation in HHT Patients


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Edwards, C.P., Shehata, N. & Faughnan, M.E. Hereditary hemorrhagic telangiectasia patients can tolerate anticoagulation. Ann Hematol 91, 1959–1968 (2012).

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