Correspondence
Efficacy of Unusually High Doses of Tranexamic Acid for the Treatment of Epistaxis in Hereditary Hemorrhagic Telangiectasia
N Engl J Med 2001; 345:926September 20, 2001
- Article
To the Editor:
Hereditary hemorrhagic telangiectasia is an autosomal dominant disease characterized by epistaxis, cutaneous telangiectases, and visceral arteriovenous malformations.1 Aminocaproic acid, an antifibrinolytic drug, can reduce epistaxis in hereditary hemorrhagic telangiectasia,2 but its effect is inconsistent.3 We successfully treated three patients who had hereditary hemorrhagic telangiectasia with tranexamic acid, another antifibrinolytic drug.
The first patient was a 69-year-old man who received 1 g of tranexamic acid four times daily. After three months of therapy, he reported an improvement in his epistaxis and his hemoglobin level rose from 10.3 g per deciliter to 12.3 g per deciliter. The second patient was a 65-year-old man who had uncontrolled epistaxis despite two embolizations of the internal maxillary arteries. Three months after the start of therapy with 1 g of tranexamic acid four times daily, his hemoglobin level increased from 7.7 g per deciliter to 12.9 g per deciliter. The third patient was a 42-year-old man who received 500 mg of tranexamic acid every two hours (total, 4.5 g daily). The frequency of his epistaxis decreased from an average of 120 episodes per month before tranexamic acid therapy was initiated to an average of 60 episodes per month after the initiation of treatment. His hemoglobin level rose from 9 g per deciliter to 12 g per deciliter. All three patients received iron therapy both before and during treatment with tranexamic acid. No blood transfusions were administered during therapy with tranexamic acid, and no adverse effects related to therapy were observed.
Kwaan and Silverman4 demonstrated local hyperfibrinolysis in lesions due to hereditary hemorrhagic telangiectasia mediated by an increase in tissue plasminogen activator. This finding provides a rational basis for the use of antifibrinolytic drugs in the disease. We used tranexamic acid, an antifibrinolytic drug that is 10 times as potent as aminocaproic acid and has a longer half-life. Recently, success has also been reported with the use of topical tranexamic acid for severe epistaxis.5 Our finding that therapy with tranexamic acid controlled epistaxis in patients with hereditary hemorrhagic telangiectasia is encouraging and suggests a need for prospective clinical trials with this agent.
5 ReferencesCarlo Sabbà, M.D.
Mauro Gallitelli, M.D.
Giuseppe Palasciano, M.D.
Università di Bari, 70124 Bari, Italy
c.sabba@dimimp. uniba. it 1
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Andrea Penaloza, Marie-Christiane Vekemans, Catherine Lambert, Cedric Hermans. (2011) Deep vein thrombosis induced by thalidomide to control epistaxis secondary to hereditary haemorrhagic telangiectasia. Blood Coagulation & Fibrinolysis 22:7, 616-618
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Jonathan Joseph, Pablo Martinez-Devesa, Rafael Perera, Jonathan Joseph. 2010. Antifibrinolytic agent tranexamic acid for nasal haemorrhage (epistaxis). .
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Claire L. Shovlin. (2010) Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment. Blood Reviews 24:6, 203-219
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Carmelo Bernabéu, Francisco Javier Blanco, Carmen Langa, Eva María Garrido-Martin, Luisa María Botella. (2010) Involvement of the TGF-β superfamily signalling pathway in hereditary haemorrhagic telangiectasia. Journal of Applied Biomedicine 8:3, 169-177
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S. DUPUIS-GIROD, S. BAILLY, H. PLAUCHU. (2010) Hereditary hemorrhagic telangiectasia: from molecular biology to patient care. Journal of Thrombosis and Haemostasis 8:7, 1447-1456
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Helen M. Shields, Kitt Shaffer, Richard P. O’Farrell, Robin Travers, Jane N. Hayward, Laren S. Becker, Gregory Y. Lauwers. (2007) Gastrointestinal Manifestations of Dermatologic Disorders. Clinical Gastroenterology and Hepatology 5:9, 1010-1017
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Carmelo Morales-Angulo, Alfonso Pérez del Molino, Roberto Zarrabeitia, África Fernández, Francisco Sanz-Rodríguez, Luisa María Botella. (2007) Tratamiento de las epistaxis en la telangiectasia hemorrágica hereditaria (enfermedad de Rendu-Osler-Weber) con ácido tranexámico. Acta Otorrinolaringológica Española 58:4, 129-132
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Ljiljana Jovancevic, Slobodan Mitrovic. (2006) Epistaxis in patients with hereditary hemorrhagic teleangiectasia. Medicinski pregled 59:9-10, 443-449
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