Treatment of Bleeding in Hereditary Hemorrhagic Telangiectasia with Aminocaproic Acid

Hi, Saba; Ga, Morelli; La, Logrono

doi:10.1056/nejm199406233302504

Cited by 93 publications

(43 citation statements)

References 26 publications

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“…Therapeutic manipulation of coagulation and fibrinolytic pathways is often employed to try to limit blood loss in HHT 41,42,181,182 . These therapies have not yet been supported by data from randomized controlled trials.…”

Section: Antifibrinolytics and Prothrombotic Agentsmentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects approximately 1 in 5,000 people. The abnormal vascular structures in HHT result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms underlying the generation of HHT telangiectasia and arteriovenous malformations are being unravelled, with recent data focussing on a defective response to angiogenic stimuli in particular settings. For affected individuals, there is often substantial morbidity due to sustained and repeated haemorrhages from telangiectasia in the nose and gut. Particular haematological clinical challenges include the management of severe iron deficiency anaemia; handling the intricate balance of antiplatelet or anticoagulants for HHT patients in whom there are often compelling clinical reasons to use such agents; and evaluation of apparently attractive experimental therapies promoted in high profile publications when guidelines and reviews are quickly superseded. There is also a need for sound screening programmes for silent arteriovenous malformations. These occur commonly in the pulmonary, cerebral, and hepatic circulations, may haemorrhage, but predominantly result in more complex pathophysiology due to consequences of defective endothelium, or shunts that bypass specific capillary beds. This review will focus on the new evidence and concepts in this complex and fascinating condition, placing these in context for both clinicians and scientists, with a particular emphasis on haematological settings. Blood Reviews _ HHT 2010_ Shovlin 3 Overview of HHTHHT, also known as Osler Weber Rendu syndrome [1][2][3] , is one of the most common disorders to be inherited as an autosomal dominant trait. Careful epidemiological studies reveal that it affects approximately 1 in 5,000 individuals, 4,5 with regional differences, 6 and isolated communities displaying higher prevalences due to founder effects. 7 8 HHT was first described as a familial disease characterised by severe recurrent nasal and gastrointestinal bleeding with associated anaemia, and visible dilated blood vessels (telangiectasia) on the lips and finger tips. The majority of HHT patients are also affected by larger arteriovenous malformations (AVMs) in the pulmonary, hepatic, cerebral, pancreatic, spinal and other circulations. 1,2,9 These features, presented in more detail within Table 1, are used as criteria to diagnose HHT. 2 The spectrum of disease within the HHT umbrella has extended beyond the telangiectatic/AVM HHT pathology delineated by the Curaçao criteria. 2 More recently recognised features include pulmonary arterial hypertension 10 ; juvenile polyposis 11 ; pulmonary hypertension in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [12][13][14][15][16] ; a prothrombotic state associated with elevated plasma levels of factor VIII 17 , and poten...

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Section: Antifibrinolytics and Prothrombotic Agentsmentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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“…There is only one isolated report in which it was effective in the management of epistaxis from arteriovenous malformations in two patients with HHT at a dose of 1.5 g twice a day, although it was not clear whether concomitant gastrointestinal bleeding was present. 39 Tranexamic acid is a synthetic lysine analog that inhibits the conversion of plasmin to fibrinogen, with less antifibrinolytic power than aminocaproic acid. Tranexamic acid has been proven useful for chronic bleeding from angiodysplasias in patients with end-stage renal failure and bleeding gastric antral vascular ectasia in cirrhosis.…”

Section: Antifibrinolyticsmentioning

confidence: 99%

Pharmacological Therapy for Recurrent Obscure Gastrointestinal Bleeding

Molina–Infante¹,

Vinagre-Rodríguez²,

Fernandez–Bermejo³

2011

Gastrointestinal Endoscopy

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“…There have been reports of its effectiveness in the management of bleeding in patients with and without vWF deficiency [52]. Epsilon (e) aminocaproic acid (EACA), a potent inhibitor of the fibrinolytic system, has been reported to decrease the frequency of bleeding episodes and the number of transfusions required, and to stabilize hemoglobin values in patients treated long term [53,54]. Patients should be advised to avoid NSAIDs, coumadin and potassium chloride which might increase the risk of gastrointestinal bleeding.…”

Section: Gastrointestinal Involvementmentioning

confidence: 99%

Gastrointestinal and Hepatic Manifestations of Hereditary Hemorrhagic Telangiectasia

Sharma

Howden

1998

Dig Dis

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Hereditary hemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber (ORW) disease is an autosomal-dominant disorder that frequently presents with gastrointestinal bleeding which may be a diagnostic and therapeutic challenge. Hepatic involvement in this disease is increasingly recognized and poses another therapeutic challenge. With advances in genetic screening and diagnostic procedures, and the increasing awareness of the condition by physicians and patients, this disease is being diagnosed more often. This article reviews the available literature on gastrointestinal and hepatic manifestations of HHT, and the various diagnostic and therapeutic modalities available for its management.

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Treatment of Bleeding in Hereditary Hemorrhagic Telangiectasia with Aminocaproic Acid

Cited by 93 publications

References 26 publications

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Pharmacological Therapy for Recurrent Obscure Gastrointestinal Bleeding

Gastrointestinal and Hepatic Manifestations of Hereditary Hemorrhagic Telangiectasia

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