Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - a double-blind placebo-controlled cross-over phase IIIB study

Geisthoff, Urban W.; Seyfert, U. T.; Kübler, Marcus; Bieg, Birgitt; Plinkert, Peter K.; König, J.

doi:10.1016/j.thromres.2014.06.012

Cited by 79 publications

(94 citation statements)

References 21 publications

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“…26 Tranexamic acid has been shown to be modestly effective in reducing epistaxis frequency and severity in patients with HHT in randomized controlled trials. 8,33 Thermal and chemical coagulations are effective but less favored because of higher rates of complications, such as scarring and septal perforations. Endoscopic application of potassium titanyl phosphate or neodymium-doped yttrium aluminium garnet laser has been shown to be effective but repeated applications are necessary to maintain benefit.…”

Section: Epistaxismentioning

confidence: 99%

“…Patients with HHT often present with a wide range of serious neurological, pulmonary, and gastrointestinal complications, such as cerebral and spinal AVMs, cerebral abscess, stroke, epistaxis, pulmonary AVMs (PAVMs), liver AVMs, and gastrointestinal bleeding. [2][3][4][5][6][7][8] HHT is diagnosed clinically using the Curacao criteria. 9 The 4 Curacao criteria are (1) spontaneous and recurrent epistaxis, (2) mucocutaneous telangiectasias (lips, oral cavity, face, and fingers) (3) visceral AVMs (brain, liver, lung, etc.…”

mentioning

confidence: 99%

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Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia

Brinjikji

Iyer

Sorenson

et al. 2015

Stroke

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Section: Epistaxismentioning

confidence: 99%

mentioning

confidence: 99%

Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia

Brinjikji

Iyer

Sorenson

et al. 2015

Stroke

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“…61 In a randomized, double-blind, placebo-controlled, crossover study of 22 patients, tranexamic acid 1 g 3 times daily resulted in a 54% reduction in nosebleeds while on tranexamic acid as compared with the placebo treatment period, although there was no statistically significant improvement in hemoglobin concentration. 62 Apart from its inhibition of plasmin, tranexamic acid may have some effect on the underlying disease process; it appears to increase endoglin and ALK1 levels on the endothelium, selectively stimulating the TGF-β pathway. 63 Tranexamic acid may have a higher potency and longer half-life than aminocaproic acid in these patients.…”

Section: Assessment and Managementmentioning

confidence: 99%

Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist’s perspective

2018

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Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder that causes abnormal blood vessel formation. The diagnosis of hereditary hemorrhagic telangiectasia is clinical, based on the Curaçao criteria. Genetic mutations that have been identified include ENG, ACVRL1/ALK1, and MADH4/SMAD4, among others. Patients with HHT may have telangiectasias and arteriovenous malformations in various organs and suffer from many complications including bleeding, anemia, iron deficiency, and high-output heart failure. Families with the same mutation exhibit considerable phenotypic variation. Optimal treatment is best delivered via a multidisciplinary approach with appropriate diagnosis, screening and local and/or systemic management of lesions. Anti-angiogenic agents such as bevacizumab have emerged as a promising systemic therapy in reducing bleeding complications but are not curative. Other pharmacological agents include iron supplementation, antifibrinolytics and hormonal treatment. This review discusses the biology of HHT, management issues that face the practising hematologist, and considerations of future directions in HHT treatment.

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“…A small randomised placebocontrolled double-blind trial showed a significant reduction in epistaxis over six months as measured by patient-reported epistaxis scores (75) . In vitro cultures suggest that tranexamic acid acts by stimulating the TGF-b /ALK1/endoglin pathway.…”

Section: Antifibrinolytic Agentsmentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia

Rimmer

Lund

2015

Rhin

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Background: Hereditary haemorrhagic telangiectasia is an autosomal dominant vascular disease characterized by recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. Methodology:The genetic basis and pathophysiology of the disease are discussed. Diagnostic criteria and the clinical course of the condition are considered. The current management options, both medical and surgical, are reviewed. Conclusions:Hereditary haemorrhagic telangiectasia requires specialist treatment for the problems it causes, and is best managed in specialist centres. Epistaxis is often the major symptom, significantly affecting patients' quality of life. An understanding of the available treatment options is therefore important for the otorhinolaryngologist.

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Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - a double-blind placebo-controlled cross-over phase IIIB study

Cited by 79 publications

References 21 publications

Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia

Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist’s perspective

Hereditary haemorrhagic telangiectasia

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