Long-term outcome of chronic hepatitis C virus infection in primary hypogammaglobulinaemia

The clinical course of HCV infection in patients with primary hypogammaglob ulinaemia appears to be more severe than in immunocompetent patients. We st udied the long-term course of chronic HCV infection in 20 Norwegian hypogam maglobulinaemia patients with a 13-15 year known history of HCV infection. Twelve of 20 patients developed cirrhosis during the observation period (19 84-1999), and the remaining eight also had chronic liver disease verified b y liver biopsy in the majority of the cases. Eleven of the 20 patients are dead. Two died following liver transplantation for HCV cirrhosis. Five died due to terminal liver failure without receiving a liver allograft. Two pat ients died from other causes, but with advanced liver disease contributing to the outcome, while two deaths were unrelated to the HCV infection. Among patients with common variable immunodeficiency (CVI), five out of six are dead. Two patients cleared the hepatitis C virus 3 years following interfer on monotherapy, while three patients achieved a sustained response to combi nation therapy with interferon and ribavirin. Viral load did not seem to ha ve a major impact on disease progression. Our results emphasize the severit y of hepatitis C virus infection in patients with hypogammaglobulinaemia. P atients with CVI appear to have the poorest prognosis.