The management of liver disease in people with congenital bleeding disorders: guidance from European Association for Haemophilia and Allied Disorders, European Haemophilia Consortium, ISTH, and World Federation of Hemophilia.
Vincenzo La Mura 1), 2); Massimo Colombo 3); Graham R. Foster 4); Paolo Angeli 5); Wolfgang Miesbach 6); Robert Klamroth 7); Glenn F. Pierce 8); Brian O’Mahony 9), 10); Ming Y. Lim 11); Virginia Hernandez-Gea 12); Michael Makris 13); Flora Peyvandi 1), 2)
1) Fondazione Istituto Ricovero e Cura a Carattere Scientifico (I.R.C.C.S.) Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi and Thrombosis Center, Milan, Italy
2) Department of Pathophysiology and Transplantation (DEPT), University of Milan, Milan, Italy
3) European Association for the Study of the Liver (EASL) International Liver Foundation, Geneva, Switzerland
4) Barts Liver Centre, Blizard Institute, Queen Mary University of London (QMUL), London, United Kingdom
5) Unit of Internal Medicine and Hepatology (UIMH), Department of Medicine (DIMED), University of Padova, Padova, Italy
6) Department of Haemostasis/Haemophilia Centre, Medical Clinic 2, University Hospital Frankfurt, Frankfurt, Germany
7) Department for Internal Medicine and Vascular Medicine, Haemophilia Treatment Center, Vivantes Hospital im Friedrichshain, Berlin, Germany
8) World Federation of Hemophilia (WFH), Montreal, Quebec, Canada
9) Irish Haemophilia Society, Dublin, Ireland
10) Trinity College Dublin, Dublin, Ireland
11) Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, University of Utah, Salt Lake City, Utah, USA
12) Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica Red de Enfermedades Hepáticas y Digestivas (CIBEREHD), Care Provider of the European Reference Network on Rare Liver Disorders (ERN-RareLiver), Universitat de Barcelona, Barcelona, Spain
13) School of Medicine and Population Health, University of Sheffield, Sheffield, United Kingdom
Abstract
People with bleeding disorders (PWBD) have been exposed to the risk of developing chronic viral hepatitis and cirrhosis after replacement therapy. Today, the advent of new pharmacologic strategies for the control of hemostasis and the efficacious antiviral therapies against hepatitis C virus and hepatitis B virus have significantly reduced this risk. However, the definitive success for liver health in this clinical setting is also influenced by other factors, such as the severity of liver disease at the time of hepatitis B virus/hepatitis C virus antiviral therapy and the exposure to highly prevalent factors of chronic liver damage (eg, metabolic dysfunction and/or alcohol) that can cause a residual risk of complications such as hepatocellular carcinoma, portal hypertension, and liver insufficiency. With this background, a group of experts selected among hepatologists, hematologists, 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.