T Rosenberg1, A D Fialla2, J Kjeldsen3, A D Kjeldsen1. 1. Department of ORL-Head and Neck Surgery, Odense University Hospital, Denmark; Department of Clinical Research, University of Southern Denmark. 2. Department of Medical Gastroenterology and Hepatology, Odense University Hospital, Denmark. 3. Department of Clinical Research, University of Southern Denmark; Department of Medical Gastroenterology and Hepatology, Odense University Hospital, Denmark.
Abstract
BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disorder, with a wide variety of clinical manifestations due to the presence of multiple arteriovenous manifestations. Severe bleeding from the gastrointestinal (GI) tract and/or epistaxis presents a significant problem in a subgroup of patients and systemic bevacizumab, an angiogenesis inhibitor, has been suggested to benefit these patients. OBJECTIVE: To perform a review of the literature concerning the efficacy of systemic bevacizumab in treatment of bleeding from the nose or GI tract in patients with HHT, including patients from our own HHT-center. METHODS: A literature review was performed using the guideline "Preferred Reporting Items for systematic Reviews and MetaAnalysis statement" (PRISMA). RESULTS: After careful selection, we finally analysed the results of eight case series and 33 case reports. Among 195 patients 171 (88%) had reduced bleeding after bevacizumab. CONCLUSIONS: Based on the literature review and data from our own case series, systemic bevacizumab is very promising as treatment for HHT patients with severe epistaxis and/or GI-bleeding. However, care should be taken using bevacizumab, a potent angiogenesis inhibitor; long-term side effects have not been studied in this population. A randomized controlled study is warranted to support the results in HHT patients.
BACKGROUND:Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disorder, with a wide variety of clinical manifestations due to the presence of multiple arteriovenous manifestations. Severe bleeding from the gastrointestinal (GI) tract and/or epistaxis presents a significant problem in a subgroup of patients and systemic bevacizumab, an angiogenesis inhibitor, has been suggested to benefit these patients. OBJECTIVE: To perform a review of the literature concerning the efficacy of systemic bevacizumab in treatment of bleeding from the nose or GI tract in patients with HHT, including patients from our own HHT-center. METHODS: A literature review was performed using the guideline "Preferred Reporting Items for systematic Reviews and MetaAnalysis statement" (PRISMA). RESULTS: After careful selection, we finally analysed the results of eight case series and 33 case reports. Among 195 patients 171 (88%) had reduced bleeding after bevacizumab. CONCLUSIONS: Based on the literature review and data from our own case series, systemic bevacizumab is very promising as treatment for HHTpatients with severe epistaxis and/or GI-bleeding. However, care should be taken using bevacizumab, a potent angiogenesis inhibitor; long-term side effects have not been studied in this population. A randomized controlled study is warranted to support the results in HHTpatients.
Authors: Cristina Egido-Turrión; Elisa Rossi; Claudia Ollauri-Ibáñez; María L Pérez-García; María A Sevilla; José María Bastida; José Ramón González-Porras; Alicia Rodríguez-Barbero; Carmelo Bernabeu; José M Lopez-Novoa; Miguel Pericacho Journal: Front Med (Lausanne) Date: 2022-05-19
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