How is GI bleeding treated in Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

Nonoperative treatment of GI bleeding may include the following:

  • Estrogen-progesterone therapy
  • Transfusion
  • Aminocaproic acid
  • Endoscopic photoablation or electrocautery
  • Bevacizumab

Aminocaproic acid blocks the conversion of plasminogen to plasmin and thus acts as a powerful inhibitor of fibrinolysis. Although coagulation is thought to be normal in HHT, some investigators believe that the cause of the bleeding tendency is multifactorial. Increased plasminogen-activator activity has been demonstrated in the telangiectatic vessel walls of some patients with HHT.

A distinction is made between the malformations of HHT and lesions of angiodysplasia, both of which tend to manifest with age. The lesions of HHT are most often diffuse, and extensive surgical resection is generally not indicated for episodic bleeding, though it may be indicated for massive hemorrhage.

A treatment regimen involving intranasal administration of diluted bevacizumab has been shown to be clinically effective in patients with HHT1 and severe GI bleeding. [117]

Life-threatening GI bleeding can often be effectively treated by means of segmental bowel resection.

For additional information, see Lower GI Bleeding and Upper GI Bleeding.


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