Liver Disease and Pathology in Hemophilia

Hemophilic patients given replacement therapy with clotting factors are frequently exposed to hepatitis viruses or viral products. For the large percentage of these patients with serological evidence for past or current hepatitis B infection, the prevalence of clinically recognizable hepatitis is surprisingly low. Several prospective studies of asymptomatic patients with hemophilia have revealed that many of them have prolonged, mild‐to‐moderate elevations of serum transaminase. Liver biopsies taken from such patients provided unequivocal evidence for the existence of chronic hepatitis. Current studies of hemophiliacs with chronic hepatitis indicate that HBsAg‐positive patients may develop severe forms of liver disease, while markers of intrahepatic delta agent replication have been found only in patients with chronic active hepatitis or cirrhosis. On the contrary nonA nonB chronic hepatitis appears to be milder than expected from studies of patients undergoing open‐heart surgery. The giving of factor concentrates during the first years of life seems to be important for development of liver disease. However, the nature of the infection (type B and delta) rather than the cumulative life‐time exposure to concentrates seems to be the important element for determining the severity of the hepatic lesions.