The management of liver disease in people with congenital bleeding disorders: guidance from EAHAD, EHC, ISTH, WFH.

Abstract

People with bleeding disorders (PWBD) have been exposed to the risk of developing chronic viral hepatitis and cirrhosis after replacement therapy. At today, the advent of new pharmacological strategies for the control of hemostasis and the efficacious antiviral therapies against HCV and HBV have significantly reduced this risk. However, the real success for liver health in this clinical setting is based on other risk factors, among them, the severity of liver disease at time of HBV/HCV antiviral therapy and the exposure to highly prevalent factors of chronic liver damage (e.g.; metabolic dysfunction and/or alcohol) that can cause a residual risk of complications such as hepatocellular carcinoma, portal hypertension, liver insufficiency. With this background, a group of experts selected among hepatologists, PWBD treaters and patient representatives, produced this practical multisociety guidance for the protection of liver health and the prevention and management of liver complications in PWBD based on the most updated protocols of care.