Abstract

Hereditary haemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare, vascular, autosomal dominant disorder. The purpose of this paper is to describe the efficacy and safety of treatment with intranasal bevacizumab in HHT.

A 42-year-old woman with HHT presented with frequent episodes of epistaxis. Iron studies showed anaemia of iron deficiency from chronic blood loss. Because of the frequent epistaxis (Epistaxis Severity Score (ESS) 6.76) and varying haemoglobin levels (Hb range: 7.7–9.9g/dL) her doctors sought treatment with intranasal bevacizumab. This treatment was prescribed at the hospital pharmacy department in a laminar flow hood. 2.5 mL (25 mg) were placed in a nasal spray bottle. The recommended dosage was twice a day for two consecutive months. Nasal treatment seemed to control her epistaxis, and no adverse effects were reported. She only had a few further minor episodes of epistaxis, which were easily controlled (ESS 3.44). The haemoglobin levels evreached normal levels (Hb range: 12.8–14.1g/dL).