Intravenous Bevacizumab for Refractory Hereditary Hemorrhagic Telangiectasia-Related Epistaxis and Gastrointestinal Bleeding

Vivek N. Iyer, Dinesh R. Apala, Bibek S. Pannu, Aditya Kotecha, Waleed Brinjikji, Michael D. Leise, Patrick Sequeira Kamath, Sanjay Misra, Kebede Begna, Rodrigo Cartin-Ceba, Hilary M. DuBrock, Michael Joseph Krowka, Erin K. O'Brien, Rajiv K. Pruthi, Darrell R. Schroeder, Karen L. Swanson

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Abstract

Objective: To present a multiyear clinical experience with intravenous bevacizumab for the management of severe gastrointestinal bleeding and/or epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). Patients and Methods: All patients treated with intravenous bevacizumab for severe hereditary hemorrhagic telangiectasia-related bleeding from June 1, 2013, through January 31, 2017, were included in this report. Severity of epistaxis (determined using the Epistaxis Severity Score questionnaire); hemoglobin, iron, and ferritin levels; and quality of life data were collected serially in all patients. Results: Intravenous bevacizumab was administered to 34 patients using a standardized treatment protocol. Anemia was primarily related to severe epistaxis (n=15, 44%), severe gastrointestinal bleeding (n=4, 12%), or both (n=15, 44%), with a median baseline hemoglobin level of 9.1 g/dL (range, 8.3-10.5 gm/dL; to convert to mmol/L, multiply by 0.62). Red blood cell (RBC) transfusions had been administered to 28 patients (82%). Of these, 16 patients (47%) were RBC transfusion dependent and had received a median of 75 RBC transfusions (range, 4->500 RBC units) before bevacizumab initiation. The median length of follow-up was 17.6 months from the beginning of bevacizumab treatment (range, 3-42.5 months). There was a significant reduction in epistaxis severity scores (P<.001) and RBC transfusion requirements (P=.007) after completion of the initial bevacizumab treatment cycle. New-onset or worsened hypertension was noted in 4 patients, with 1 patient experiencing hypertensive urgency with a temporary decline in renal function. Conclusion: Intravenous bevacizumab is an effective treatment option for patients with severe anemia related to epistaxis and/or gastrointestinal bleeding. Further studies are needed to establish a dose-response relationship as well as clinical, genetic, and biomarker predictors of response.

Original languageEnglish (US)
JournalMayo Clinic Proceedings
DOIs
StateAccepted/In press - Jan 1 2018

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ASJC Scopus subject areas

  • Medicine(all)

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Iyer, V. N., Apala, D. R., Pannu, B. S., Kotecha, A., Brinjikji, W., Leise, M. D., Kamath, P. S., Misra, S., Begna, K., Cartin-Ceba, R., DuBrock, H. M., Krowka, M. J., O'Brien, E. K., Pruthi, R. K., Schroeder, D. R., & Swanson, K. L. (Accepted/In press). Intravenous Bevacizumab for Refractory Hereditary Hemorrhagic Telangiectasia-Related Epistaxis and Gastrointestinal Bleeding. Mayo Clinic Proceedings. https://doi.org/10.1016/j.mayocp.2017.11.013