Bevacizumab for Refractory Gastrointestinal Bleeding in Rendu-Osler-Weber Disease

Abstract

Rendu-Osler-Weber disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder which is often characterized by recurrent epistaxis, mucocutaneous and gastrointestinal telangiectasias, and visceral arteriovenous malformations. Patients with gastrointestinal involvement can present with a wide spectrum of severity, which may vary from uncomplicated iron deficiency anemia to continuous and refractory bleeding. We present the case of a 62-year-old female, who was admitted with anemia following several episodes of melena, and whose endoscopic examination revealed multiple angiodysplasias in the stomach and small bowel. Despite endoscopic and medical treatment attempts with hormonal agents and octreotide, she developed persistent hemorrhage and severe anemia, requiring frequent red blood cell transfusions. Immediately after initiating bevacizumab (7.5 mg/kg, every 3 weeks), complete cessation of bleeding episodes was observed. Currently, after 1 year of follow-up, she maintained sustained remission without the occurrence of adverse events.

Safe use of systemic bevacizumab for respiratory recurrent papillomatosis in two children

“…10 More data may come from the treatment of Rendu-Osler-Weber disease, where lower doses of systemic bevacizumab are currently being used to treat refractory bleeding. 11,12 Even with the limitations of this study, we report the use of systemic bevacizumab in two children without major complications and with a positive effect on respiratory symptoms during a follow-up of 4 years and 19 months, respectively.…”

Systemic Bevacizumab (Avastin) for Juvenile‐Onset Recurrent Respiratory Papillomatosis: A Systematic Review

“…Its use has only been described in one pediatric case of HHT; it was well tolerated at 5 mg/kg every 15 days 34 . Several reports of systemic bevacizumab treatment in adults with HHT have also included smaller doses of 5 mg/kg or 7.5 mg/kg every 2 to 3 weeks compared to the 10‐mg/kg dosing used in most cases of JORRP 35–37 . There were no serious adverse events reported in these studies.…”

Diagnostic yield of capsule endoscopy for small bowel arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: a systematic review and meta-analysis

“…Of note, since the publication of these guidelines, reports on treatment of AVMs in the general population with hormonal therapies have provided mixed results, with some studies demonstrating a beneficial role in decreasing red blood cell transfusion requirements and overall rebleeding rates and others and a meta-analysis suggesting that these therapies ineffectively control bleeding 28 29 . Since the publication of these recommendations, bevacizumab, a drug extensively studied in HHT patients for treatment of epistaxis, has gained attention and is now among the most studied drugs in HHT patients with evidence of gastrointestinal bleeding in 16 case series/reports of 31 patients, four retrospective studies, and one Phase II study 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 . In these studies, bevacizumab decreased the frequency and severity of gastrointestinal bleeding and epistaxis, led to higher baseline hemoglobin levels, and reduced red blood cell transfusion requirements and dependency.…”