CASE REPORTSGastrointestinal angiodysplasia in two patients with type 3 von Willebrand diseaseLiao, Li-China; Liao, Szu-Chiab; Chang, Chung-Hsinb; Shih, Ming-Yangc; Wang, Jiaan-Derc,dAuthor Information aDepartment of Pediatrics bDivision of Gastroenterology cCenter for Rare Disease and Hemophilia, Taichung Veterans General Hospital dTunghai University, Taichung, Taiwan Correspondence to Jiaan-Der Wang, MD, PhD, Center for Rare Disease and Hemophilia, Taichung Veterans General Hospital, Taichung 40705, Taiwan Tel: +886 4 23592525 x5995; fax: +886 4 2374 1359; e-mail: [email protected] Received 23 January, 2019 Revised 15 April, 2019 Accepted 24 April, 2019 Blood Coagulation & Fibrinolysis: July 2019 - Volume 30 - Issue 5 - p 243-245 doi: 10.1097/MBC.0000000000000815 Buy Metrics Abstract Angiodysplastic (AD) lesion is the most common cause of recurrent gastrointestinal (GI) bleeding in inherited Von Willebrand disease (VWD) patients lacking high-molecular-weight multimers. Defect or dysfunction of von Willebrand factor (VWF) may lead to enhanced endothelial cell proliferation followed by the development of neoangiogenesis and vascular malformation, which result in severe bleeding. Recurrent bleeding causing by GI AD is a challenging complication of VWD. The management of VWD could be difficult due to frequent recurrence and severity of bleeding episodes. The primary aim of management is not only to stop but also to prevent bleeding. We present two patients of type 3 VWD associated with AD and severe GI bleeding, which were successfully treated by endoscopic coagulation and prophylactic therapy with different regimens of plasma-derived VWF/factor VIII (pdVWF/FVIII) concentrate to maintain a trough level in the patient unresponsive to standard treatment. Copyright © 2019 YEAR Wolters Kluwer Health, Inc. All rights reserved.