hematology and oncology
Hereditary Hemorrhagic Telangiectasia
Key Points
Nasal and gastrointestinal telangiectasias may cause significant external hemorrhage in patients with hereditary hemorrhagic telangiectasia. Vascular malformations in the central nervous system, lungs, and liver may bleed; hepatic and pulmonary malformations may cause significant shunting. Accessible mucosal telangiectasias and arteriovenous malformations may be treated with laser ablation; coil embolization or surgical resection may be needed for pulmonary vascular malformations. Antifibrinolytic drugs and angiogenesis inhibitors may reduce the incidence of bleeding. Many patients require parenteral iron supplements because of chronic blood loss. All IV solutions should be given through a filter to avoid paradoxical embolization of particulate matter to the brain through pulmonary arteriovenous malformations. 