Improvement of Ischemic Cholangiopathy in Three Patients with Hereditary Hemorrhagic Telangiectasia Following Treatment with Bevacizumab
Case Report Improvement of ischemic cholangiopathy in three patients with hereditary hemorrhagic telangiectasia following treatment with bevacizumab Paraskevi A. Vlachou1, Errol Colak1, Alexander Koculym1, Anish Kirpalani1, Tae Kyoung Kim2, ⇑ ⇑ Gideon M. Hirschfield3,4, , , Marie E. Faughnan5,6, , 1Department of Diagnostic Imaging, St. Michael’s Hospital, 30 Bond Street, Toronto, ON, Canada M5B 1W8; 2Department of Diagnostic Imaging, Toronto General Hospital, 200 Elizabeth St., Toronto, ON, Canada M5G 2C4; 3Department of Medicine, Division of Gastroenterology, University of Toronto, Toronto, Canada; 4Centre for Liver Research, Institute of Biomedical Research, NIHR Biomedical Research Unit, University of Birmingham, Birmingham B15 2TT, UK; 5Department of Medicine, Division of Respirology, Toronto HHT Centre, St. Michael’s Hospital, Toronto, Canada; 6Keenan Research Centre of the Li Ka Shing Knowledge Institute of St. Michael’s Hospital, 30 Bond Street, Toronto, ON, Canada M5B 1W8 Abstract sias and visceral arteriovenous malformations [1]. Although radiological signs of liver involvement occur in more than 70% The ischemic biliary phenotype of hereditary hemorrhagic telan- of patients, less than 10% of these patients develop symptoms giectasia (HHT) is rare but distinct, with progressive biliary tree [2]. Symptomatic liver HHT from intrahepatic shunting can lead ischemia usually resulting in an irreversible secondary sclerosing to patients presenting with high-output cardiac failure (HOCF), cholangiopathy. When clinically severe, liver transplant is often portal hypertension or ischemic biliary disease [3,4]. A recent indicated. We report three patients with marked HHT Italian prospective cohort study found that over prolonged fol- associated biliary disease, in whom prolonged anti-vascular low-up, substantial morbidity and mortality were associated endothelial growth factor therapy (bevacizumab) notably with liver vascular malformations in HHT patients [5].
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