Background: Hereditary hemorrhagic telangiectasia (HHT) is a multisystem disease that is marked by mutations regulating vasculature formation. Epistaxis is the most commonly reported symptom, but gastrointestinal bleeding, anemia, hepatic issues, and pulmonary disease are also common. There is a growing body of evidence in the literature concerning using the monoclonal antibody against vascular endothelial growth factor (VEGF), bevacizumab, in patients with HHT. This treatment is gaining support for managing HHT because it directly inhibits the VEGF proteins that can be elevated as a result of the HHT mutations. We reviewed the current literature on the outcomes from intravenous bevacizumab treatment for HHT with a focus on epistaxis outcomes.
Methods: A systematic review of the literature was performed using Ovid MEDLINE, Scopus, and Cochrane databases. English citations, both national and international, were reviewed and filtered for relevance.
Results: Eighteen studies were included in this review. The majority of citations were case reports. All studies reported improvements. Specifically, 14 reported improvements in epistaxis, and 11 reported hemoglobin improvement following intravenous (IV) bevacizumab. Lack of uniformity in data presentation prevented a meta-analysis.
Conclusion: This is the first systematic review analyzing the data involving HHT patients treated with bevacizumab. The results show that patients treated with bevacizumab have global improvements as well as specific improvements in hemoglobin levels. Although all of the studies reported improvements, there are several limitations, including inconsistencies in outcome reporting. A large, randomized, controlled study is needed to further investigate hemorrhage and epistaxis outcomes in HHT patients treated with intravenous bevacizumab.
Keywords: VEGF; anemia; bevacizumab; epistaxis; hemorrhage; hereditary hemorrhagic telangiectasia.
© 2015 ARS-AAOA, LLC.