Background Hereditary haemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease, is a rare, vascular, autosomal dominant disorder. Telangiectasias and arteriovenous malformations (AVMs) of the lung, liver and CNS are vascular lesions present in HHT, most commonly causing epistaxis and gastrointestinal bleeding. The diagnosis is based on the Curaçao criteria. Recently, the epistaxis severity score (ESS) was created as a standardised measure to estimate the degree of epistaxis.
Purpose To describe the effectiveness and safety of treatment with intranasal bevacizumab in HHT.
Material and methods A 42-year-old woman with HHT presented with the chief complaint of frequent episodes of epistaxis. She had undergone gingival mucosa cauterisation 10 years ago and it resolved oral bleeding. Iron studies showed anaemia of iron deficiency from chronic blood loss. Initially her anaemia was treated with oral ferroproteinsuccinylate. This treatment failed; consequently, physicians replaced her treatment with intravenous iron. She received 4 blood transfusions in 2 years. Arterial embolisation was carried out in February 2015; it was unsuccessful.
Results Because of the frequent epistaxis (ESS 6.76) and varying haemoglobin (Hb) levels (Hb range 7.7–9.9) her physicians sought treatment with intranasal bevacizumab. This treatment was prepared at the hospital pharmacy department with Avastin 400 mg/16 mL vial in a laminar flow hood. Placed in a nasal spray bottle were 2.5 mL (25 mg). Each bottle was discontinued after 21 days; physicochemical properties were stable during the treatment period (21 days). The dosage was given twice a day for 2 consecutive months. Nasal treatment seemed to control her epistaxis and no adverse effects were reported. She had only a few minor episodes of epistaxis, which were easily controlled. Hb reached normal levels (Hb range 12.8–14.1) and currently iron treatment is not necessary.
Conclusion Vascular endothelial growth factor is a key pathogenic factor that acts to increase and maintain vascular density. To avoid the systemic adverse effects of bevacizumab, intranasal treatment, by either submucosal injection or topical nasal spray, has recently been reported to be a safe alternative to intravenous injection for nose bleeds.
Intranasal bevacizumab is an effective and safe treatment for severe epistaxis in patients with HHT. This therapy reduces epistaxis severity and frequency.
No conflict of interest
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