European Reference Network for Rare Vascular Diseases (VASCERN): When and how to use intravenous bevacizumab in Hereditary Haemorrhagic Telangiectasia (HHT)?

Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular multisystemic disease that leads to epistaxis, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT prevalence is estimated at 1/6000, i.e. around 85,000 European citizens,...

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Published in:European journal of medical genetics Vol. 65; no. 10; p. 104575
Main Authors: Dupuis-Girod, Sophie, Shovlin, Claire L., Kjeldsen, Anette D., Mager, Hans-Jurgen, Sabba, Carlo, Droege, Freya, Fargeton, Anne-Emmanuelle, Fialla, Annette D., Gandolfi, Silvia, Hermann, Ruben, Lenato, Gennaro M., Manfredi, Guido, Post, Marco C., Rennie, Catherine, Suppressa, Patrizia, Sure, Ulrich, Crocione, Claudia, Blom, Ria, Botella, Luisa Maria, Brocca, Fernando, Coxall, Caroline, Druckman, Karen T., Erasme, Didier, Federici, Paolo, Grabowski, Christina, Lundgren, Mildred, Søderman, Tone, Woods, Dara, Buscarini, Elisabetta
Format: Journal Article
Language:English
Published: Elsevier Masson SAS 01-10-2022
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Summary:Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular multisystemic disease that leads to epistaxis, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT prevalence is estimated at 1/6000, i.e. around 85,000 European citizens, and is served by the European Reference Network for Rare Multisystemic Vascular Diseases (VASCERN). HHT treatments depend on clinical manifestations, and span multiple different medical, surgical and interventional disciplines. Separate to local treatments in the nose, in severe settings, intravenous bevacizumab has been proposed as treatment option, and the purpose of the current article is to assess the use of intravenous bevacizumab in patients with HHT in 2022 according to available data.
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ISSN:1769-7212
1878-0849
DOI:10.1016/j.ejmg.2022.104575