A new collaborative publication by the Hereditary Haemorrhagic Telangiectasia Working Group (HHT-WG) entitled Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia has been published in the Orphanet Journal of Rare Diseases.
It is co-authored by Prof Elisabetta Buscarini (HHT-WG Deputy Co-Chair), Dr. Luisa Maria Botella, Prof. Urban Geisthoff, Prof. Anette D. Kjeldsen, Dr. Hans Jurgen Mager, Prof. Fabio Pagella, Dr. Patrizia Suppressa, Dr. Roberto Zarrabeitia, Dr. Sophie Dupuis-Girod (HHT-WG Co-Chair), Prof. Claire L. Shovlin (HHT-WG Chair), on behalf of VASCERN-HHT
This research paper reports on the drug registry conducted by the HHT-WG that evaluated both the safety and the adverse events occurring during the treatment of HHT-related manifestations with thalidomide (TH) and bevacizumab (BZB) in patients from VASCERN HHT Centers of Reference. TH and BZB are two types of anti-angiogenic substances (substances that stop the growth of new blood vessels) that have been increasingly used in the last decade in HHT patients.
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“This study evaluated the safety of BZB and TH in HHT within expert HHT centers which can offer a specific disease knowledge, an established surveillance schedule, and an appropriate indication for the use of these drugs. Importantly, to weigh against potential benefits, both BZB and TH expose patients to the risk of severe side effects, with respective event rates of 0.40 and 0.44 AEs per HHT patient, including fatalities.
With potential increase in use of BZB and TH in HHT patients, these data support appropriate weighing of the toxicities which can arise from these drugs and the practice recommendations for their prevention and management. The risk profile for TH to BZB resulting from the data generated is helpful to share in pre-treatment counselling.”
Reference: Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia.Buscarini E, Botella LM, Geisthoff U, Kjeldsen AD, Mager HJ, Pagella F, Suppressa P, Zarrabeitia R, Dupuis-Girod S, Shovlin CL; VASCERN-HHT. Orphanet J Rare Dis. 2019 Feb 4;14(1):28. doi: 10.1186/s13023-018-0982-4.PMID: 30717761