Title

Management of Liver Disease in People with Congenital Bleeding Disorders

Authoring Organizations

International Society on Thrombosis and Haemostasis

World Federation of Hemophilia

Publication Month/Year

September 11, 2024

Last Updated Month/Year

September 22, 2024

Document Type

Consensus

Country of Publication

Global

Document Objectives

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.

Inclusion Criteria

Male, Female, Adolescent, Adult, Child, Older adult

Health Care Settings

Ambulatory, Hospital, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Management

Diseases/Conditions (MeSH)

D008107 - Liver Diseases, D006467 - Hemophilia A, D002836 - Hemophilia B

Keywords

cirrhosis, fibrosis, hepatitis, hepatocellular carcinoma, hemophilia, gene therapy, Congenital Bleeding Disorders

Source Citation

LA Mura V, Colombo M, Foster GR, Angeli P, Miesbach W, Klamroth R, Pierce GF, O'Mahony B, Lim MY, Hernandez-Gea V, Makris M, Peyvandi F. The management of liver disease in people with congenital bleeding disorders: guidance from EAHAD, EHC, ISTH, WFH. J Thromb Haemost. 2024 Sep 11:S1538-7836(24)00533-6. doi: 10.1016/j.jtha.2024.08.018. Epub ahead of print. PMID: 39271019.