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

Systemic bevacizumab for the treatment of chronic bleeding in hereditary haemorrhagic telangiectasia

“…Bevacizumab was well tolerated in our study. The primary treatment‐related adverse event was hypertension in 15% of patients, a rate similar to the two aforementioned studies in HHT (which found rates of 11% and 12% ) and large trials in oncology patients . No major adverse events associated with bevacizumab in the oncology population, such as venous thromboembolism or bowel perforation, were observed.…”

“… Guilhem and colleagues retrospectively examined outcomes of 46 patients receiving short‐term systemic bevacizumab (median treatment duration approximately 3 months) for management of high‐output heart failure (ten patients), severe haemorrhage (20 patients) or both (16 patients) . Whilst including many patients, the only efficacy measure reported was the ‘clinical appreciation of efficacy by the referent physician’, reporting that 74% of all patients were subjectively improved as assessed by their physician. Iyer and colleagues retrospectively examined outcomes of 34 patients receiving a 6‐month bevacizumab treatment course (with additional treatments administered as needed for recurrent bleeding), finding significant reductions in epistaxis severity scores and red cell transfusion requirements and improvements in quality of life (as assessed by a two‐question investigator‐designed questionnaire) . Whilst supportive of the efficacy of systemic bevacizumab for HHT‐associated bleeding, posttreatment haemoglobin concentrations were not given and no iron infusion data were reported (except to note that 41% of patients had received some IV iron supplementation in the 6 months prior to bevacizumab initiation). …”

“…2 Iyer and colleagues retrospectively examined outcomes of 34 patients receiving a 6-month bevacizumab treatment course (with additional treatments administered as needed for recurrent bleeding), finding significant reductions in epistaxis severity scores and red cell transfusion requirements and improvements in quality of life (as assessed by a two-question investigatordesigned questionnaire) [23]. Whilst supportive of the efficacy of systemic bevacizumab for HHTassociated bleeding, posttreatment haemoglobin concentrations were not given and no iron Haemoglobin(g dL -1 ) Fig.…”

Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist’s perspective

“…describes a large cohort of HHT patients receiving bevacizumab to treat GI bleeding and epistaxis. 8 Thirty-four patients were given intravenous bevacizumab according to a standardized protocol, resulting in a statistically significant reduction in epistaxis severity scores and RBC transfusion requirements, although 4 patients developed new-onset or worsened hypertension. Most published studies have used bevacizumab at a dose of 5-10 mg/kg every 2-4 weeks for up to 6 cycles.…”

“…6,7 However, data on intranasal bevacizumab have been conflicting, and studies investigating the use of intravenous bevacizumab are limited to case reports and retrospective series. 8 Treatment of HHT involves a multidisciplinary approach of specialists in cardiology, pulmonology, hepatology, interventional radiology, ear, nose and throat (ENT), genetics, and hematology. This review focuses on the biology of HHT and the management issues that confront the hematologist, as well as proposing a hematology management scheme.…”