Type III von Willebrand's disease(vWD3) is a rare hereditary hemorrhagic disorder whose incidence ranges between 0.5 and 5 cases per million population.(1) vWD3 is characterized by complete lack of von Willebrand's factor(vWF) -a platelet adhesive protein secreted by vascular endothelium- resulting in severely prolonged bleeding time.(1) A previous Italian study reported that up to 39% of vWD patients are infected with hepatitis-C virus(HCV) with an 11% incidence of advanced liver disease and 2% hepatocellular carcinoma(HCC).(2) So far, the risk for major bleeding and HCV recurrence discouraged liver transplantation(LT) and only three cases were reported with poor long-term survival due to HCV or HCC recurrence. (1,3,4) This article is protected by copyright. All rights reserved.
Liver transplantation in type III von Willebrand disease
MARENCO, SIMONA;PICCIOTTO, ANTONINO;
2015-01-01
Abstract
Type III von Willebrand's disease(vWD3) is a rare hereditary hemorrhagic disorder whose incidence ranges between 0.5 and 5 cases per million population.(1) vWD3 is characterized by complete lack of von Willebrand's factor(vWF) -a platelet adhesive protein secreted by vascular endothelium- resulting in severely prolonged bleeding time.(1) A previous Italian study reported that up to 39% of vWD patients are infected with hepatitis-C virus(HCV) with an 11% incidence of advanced liver disease and 2% hepatocellular carcinoma(HCC).(2) So far, the risk for major bleeding and HCV recurrence discouraged liver transplantation(LT) and only three cases were reported with poor long-term survival due to HCV or HCC recurrence. (1,3,4) This article is protected by copyright. All rights reserved.
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