The natural history of hepatitis C virus infection in Italian patients with von Willebrand's disease: a cohort study.

BACKGROUND AND OBJECTIVES: Unlike patients with hemophilia, those with von Willebrand's disease (VWD) have a mild to moderate bleeding tendency requiring a lower rate of transfusion: moreover, the use of blood products in most Italian patients with VWD was greatly reduced following the introduction of desmopressin in 1977. The main objective of this study was to compare the prevalence and outcome of hepatitis C virus (HCV) infection in multi-transfused patients with VWD and in those with hemophilia A or B.

DESIGN AND METHODS: In a large cohort of 356 patients with VWD (41% type 1, 53% type 2 and 6% type 3) and 340 with hemophilia A (85%) or B (15%), all of whom were negative for human immunodeficiency virus (HIV) serum HCV markers, liver function tests and abdominal ultrasound were performed every 6 months for 6 years.

RESULTS: VWD patients were less often transfused with any type of blood products than were hemophiliacs (40% versus 96%) and were, therefore, less frequently infected with HCV (39% versus 82%). HCV infection in patients with VWD occurred at an older mean age (22 versus 7 years), was of shorter duration (20 versus 31 years), and manifested less often with elevated transaminases (58% versus 83%). The risk of infection by HCV genotype 1a was significantly lower in patients with VWD than in hemophiliacs. Despite these differences in the features of HCV infection, the cumulative incidences of advanced liver disease (11% versus 10 %) and hepatocellular carcinoma (2% versus 4%) were very similar in the two groups of patients.

INTERPRETATION AND CONCLUSIONS: VWD patients had lower prevalences of HCV infection with HCV and genotype 1a infections than did hemophiliacs, reflecting the different source and type of transfused blood products. HCV infection in both groups seems to run a relatively mild course, until now, but the high prevalence of genotypes resistant to curative antiviral therapies is of concern.