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Bevacizumab Therapy Effective for Hereditary Hemorrhagic Telangiectasia

by Sue Pondrom •

Does bevacizumab delivered as a submucosal and topical intranasal therapy effectively control hereditary hemorrhagic telangiectasia (HHT)-associated epistaxis?

Background: HHT is an autosomal dominant disease with affected patients developing arteriovenous malformations that are prone to bleeding. For moderate and severe cases, epistaxis can be severe and life threatening. HHT patients have been found to have dysfunction in the TGF-β vascular endothelial growth factor (VEGF) signaling cascade.

Study design: Prospective institutional review board-approved study.

Setting: Division of Otolaryngology-Head and Neck Surgery, University of California, San Diego School of Medicine.

Synopsis: For the past five years, the researchers have been treating individuals with moderate to severe epistaxis with a 100-mg intranasal submucosal injection of bevacizumab, a VEGF inhibitor.

For this article, they reported the one-year follow-up of 19 patients treated with 100 mg of intranasal submucosal bevacizumab with or without topical bevacizumab using the epistaxis severity score (ESS) as a barometer for treatment response.

ESS data were recorded for all patients for the first nine months, and for 17 patients in months 10 to 12. Six of the 19 patients received eight additional topical bevacizumab treatments with a 100-mg bevacizumab nasal spray because their bleeding had increased. The mean preinjection ESS for the 19 patients was 8.12, with an ESS nadir of 2.00 reached at two months following submucosal injection. Over the 12 months, the mean ESS steadily increased back to a maximum of 3.6, reached at 11 months.

Bottom line: Bevacizumab is effective in the treatment of HHT epistaxis. For some, topical bevacizumab best treats patients with an ESS below five, and a more advanced disease of five or greater is initially treated with a submucosal injection, followed by topical treatment.

Reference: Karnezis TT, Davidson TM. Treatment of hereditary hemorrhagic telangiectasia with submucosal and topical bevacizumab therapy. Laryngoscope. 2012;33(12):495-497.