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Bevacizumab as therapeutic option for recurrent nasal bleeding in hereditary hemorrhagic telangiectasia.



Rok publikování 2019
Druh Další prezentace na konferencích
Fakulta / Pracoviště MU

Lékařská fakulta

Popis Introduction Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is rare autosomal dominant genetic disorder of blood vessels. This disease is characterised by telangiectasias (small vascular malformations), which are found in skin or mucosa and have high tendency to rupture. In typical cases it results in epistaxis or gastrointestinal bleeding. Epistaxis is main complaint in 90 to 96% of patients with HHT. Some patients are resistant to existing treatment options (laser rhinosurgery or septoplasty/ septodermoplasty) and often require blood transfers because of severe anemia. Vascular endothelial growth factor (VEGF) is well known angiogenic factor, which elevation is found in HHT. Bevacizumab is recombinant monoclonal antibody to VEGF, which inhibates VEGF efect. Bevacizumab can be used in patients with HHT by systemic or local application to nasal or oral mucosa to reduce frequency, duration and severity of epistaxis. Material and methods We recorded patient who was treated by topical submucous injection of bevacizumab 25mg to each side of nasal septum (Avastin 2x5ml) under the general anesthesia. This patient had long history of severe recurrent epistaxis leading to sideropenic anemia requiring blood transfusions periodically. Rhinosurgical procedures and more than 1 year topical bevacizumab application as nasal spray were without significant effect. Results We detect significant clinical effect of submucosal bevacizumab application in our patient. Frequency of epistaxis is reduced from every 3-7 days to once a month. Reduction of frequency and severity of nasal bleeding also leads to the significal improvement of quality of patient’s life. Conclusion Submucous injection of bevacizumab should be considered as an alternative treatment option, especially for refractory and severe cases of hereditary hemorrhagic telangiectasia.

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