Bevacizumab in hereditary hemorrhagic telangiectasia‐associated epistaxis: Effectiveness of an injection protocol based on the vascular anatomy of the nose

Abstract

Objective/Hypothesis:To evaluate the effectiveness of a standardized intranasal bevacizumab injection in treating hereditary hemorrhagic telangiectasia (HHT)‐associated epistaxis.Study Design:Prospective pilot study.Methods:A total dose of 100 mg bevacizumab (25 mg/mL Avastin) was injected submucosally, 50 mg on each side. A total of 0.5 mL was injected in the sphenopalatine area, upper part of bony septum, upper part of the later nasal wall, and the anterior part of nasal floor. No cauterizations or laser therapy were done during or after the procedure. The hemoglobin level and grades of epistaxis were recorded before and monthly after the procedure. The IFT grading system (intensity [I], frequency [F] of epistaxis, and the amount of blood transfusion [T]) and epistaxis severity score (ESS) for hereditary hemorrhagic telangiectasia system were used. Quality of life (QoL) was evaluated before and 4 weeks after the procedure using the Short Form‐36 Health Survey questionnaire, Cantril's Self‐Anchoring Ladder questionnaire, and Slotosch disease‐specific QoL questionnaire.Results:A significant improvement was found in IFT grading (P = .007), ESS grading (P = .001), and hemoglobin level (P = .01). The QoL differences were statistically not significant.Conclusions:The four‐injection site technique of intranasal administration of bevacizumab is an effective treatment option in HHT‐associated epistaxis, at least on the short‐term effect. Long‐term and comparative studies are needed to further evaluate the significance of this treatment modality. Laryngoscope, 2012

Molecular Pathways: Can Activin-like Kinase Pathway Inhibition Enhance the Limited Efficacy of VEGF Inhibitors?

“…Early clinical reports suggest that patients with HHT treated with bevacizumab could experience amelioration of HHT related bleeding (57,58). Currently bevacizumab is being studied in clinical trials in HHT patients with recurrent epistaxis (59,60). …”

Bevacizumab : un nouveau succès dans la maladie de Rendu-Osler

“…Les modulateurs de l'angiogenèse, avec pour chef de file l'anticorps monoclonal anti-VEGF bevacizumab, ouvrent une nouvelle voie thérapeutique [1,2]. Dans la littérature, quelques cas cliniques et petites séries rapportent l'efficacité de cette molécule, utilisée par voie topique ou systémique, sur les épistaxis récidivantes [9,10], sur l'anémie réfractaire [6] et également sur l'atteinte hépatique compliquée d'insuffisance cardiaque à haut débit, permettant une amélioration du débit cardiaque chez 25 patients [1].…”

Hereditary haemorrhagic telangiectasia

“…However, its side effect profile includes spontaneous gastrointestinal perforation, cytopaenia, hypertension, delayed wound healing, septal perforation, weakness and haemorrhage, concerns about which have understandably limited its use for epistaxis alone; it is also expensive and long-term maintenance treatment is required as its effect is not permanent (58,79) . To try and mitigate these side effects, it has also been used intranasally, both as submucosal injections and in spray form (81)(82)(83)(84)(85)(86)(87)(88)(89)(90) . Whilst most authors report reasonable efficacy with no/minimal side effects, there have been concerns regarding the development of septal perforations following submucosal injection (81,83) .…”

“…Whilst most authors report reasonable efficacy with no/minimal side effects, there have been concerns regarding the development of septal perforations following submucosal injection (81,83) . Most authors therefore advise against injecting bevacizumab over the cartilaginous septum (81,83,84,86) . The optimum dose and route of administration have not yet been found, although extended systemic dosing intervals (three-to four-weekly) and very low dose systemic bevacizumab have been suggested (91,92) .…”