Dramatic Response to Systemic Bevacizumab Therapy in a Patient with Hereditary Hemorrhagic Telangiectasia

Abstract

Hereditary hemorrhagic telangiectasia is a rare multisystem vascular disorder. Also known as Rendu Osler syndrome, it is characterized by mucocutaneous telangiectases, epistaxis, gastrointestinal bleeding, and visceral arteriovenous malformations. A sixty-two-year old male patient was followed due to hereditary hemorrhagic telangiectasia. Because of high transfusion dependency he received bevacizumab administration at a dose of 5 mg/kg every 2 weeks, for a total of eight cycles. We achieved considerable response in terms of the dependency of transfusion after bevacizumab treatment. Serum and tissue expression of vascular endothelial growth factor is increased in patients with hereditary hemorrhagic telangiectasia. Thus, logically, vascular endothelial growth factor inhibitors could be expected to function in the therapy of hereditary hemorrhagic telangiectasia as well.

Keywords

• Hereditary Hemorrhagic Telangiectasia
• Bleeding
• Bevacizumab

Herediter Hemorajik Telenjiektazi Olgusunda Sistemik Bevacizumab Tedavisine Dramatik Yanıt

Öz

Herediter hemorajik telenjiektazi nadir görülen multisistemik vasküler bir hastalıktır. Rendu Osler sendromu olarak da bilinmekle birlikte mukokutanöz telenjiektaziler, epistaksis, gastrointestinal kanamalar ve viseral arteriovenöz malformasyonlarla karakterizedir. Altmış iki yaşında erkek hasta; herediter hemorajik telenjiektazi tanısıyla takip edilmektedir. Yoğun transfüzyon ihtiyacı olması nedeniyle hastaya bevacizumab 5mg/kg dozunda 2 haftada bir olmak üzere toplam 8 kür uygulanması sonrası transfüzyon ihtiyacında dramatik azalma görülmüştür. Herediter hemorajik telenjiektazi hastalarının serum ve dokuda bakılan vasküler endotelyal büyüme faktörü düzeylerinin artmış olduğu gösterilmiştir. Bu bağlamda vasküler endotelyal büyüme faktörü inhibitörlerinin bu hastalarda etkili olacağı düşünülmektedir.

Anahtar Kelimeler

• Herediter Hemorajik Telenjiektazi
• Kanama
• Bevacizumab

Kaynakça

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