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Liver Transplantation in Type III von Willebrand Disease
Author(s) -
Ghinolfi D.,
Cecconi N.,
Molinari A. C.,
Marenco S.,
Picciotto A.,
Paolicchi A.,
De Simone P.,
Bindi L.,
Filipponi F.
Publication year - 2016
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/ajt.13642
Subject(s) - medicine , liver transplantation , transplantation , von willebrand factor , von willebrand disease , gastroenterology , liver disease , hepatitis c , incidence (geometry) , population , coagulopathy , immunology , platelet , physics , environmental health , optics
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