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HNO 1. September 2015

Bevacizumab: an option for refractory epistaxis in hereditary haemorrhagic telangiectasia

Background: Recurrent epistaxis in hereditary haemorrhagic telangiectasia (HHT) patients significantly decreases their quality of life. Treatment in therapy refractory patients is limited although various options have been tested so far.

Case report: Herein, one patient is described that was treated for HHT for over 20 years with only intermediate benefits. As epistaxis duration and frequency increased continuously, bevacizumab 5 mg/kg was administered every 2 weeks. During the time of treatment (six doses) and up to 3 month afterwards clinical symptoms, blood pressure, cardiac output, pulmonary arterial hypertension (PAH), bleeding duration and frequency were assessed as criteria for treatment benefit.

Results: Duration and frequency of epistaxis decreased immediately after the first application resulting in reduced need of blood transfusions. After completion of six cycles, a further decrease in frequency and duration of bleeding was noted. Cardiac output and PAH decreased or remained stable, respectively, during time and after treatment. No increase in blood pressure could be found but a significant increase in heart rate was experienced after completion of all six applications. Unfortunately, the patient died due to a cerebral abscess.

Conclusion: Bevacizumab led to an improvement of HHT related epistaxis, refractory to other treatments.

Background: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary vascular disorder that causes multiple clinical symptoms of different severity. Besides recurrent epistaxis, patients are affected by gastrointestinal telangiectasia, arteriovenous malformations (AVMs), predominantly of the lung and liver, and cerebral vascular malformations [ 1 ]. Since the first description of HHT, three genes (ENG, ACVRL1, MADH4) have been identified that are associated with this disease. Mutations in these genes lead to alteration in TGF-b signaling, either by affecting the TGF-b superfamily receptor (endoglin) or the intracellular signaling (Alk 1, smad4) [ 2 ]. Genomic mutations in these proteins lead to an impaired function of endothelial driven vascularisation and angiogenesis. Dysfunction leads to thin walled and dilated vessels and thereby to haemorrhage and fibrotic remodelling. So far treatment options are limited for patients suffering from HHT symptoms. Management differs, depending if symptoms are related to local AVMs (e.g. bleeding) or are systemic results thereof.

Univ. Doz. Dr. med. univ Arno Amann, Dr. Normann Steiner, Eberhard Gunsilius, Wiener klinische Wochenschrift 15/16/2015

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