PB0211 - Prevalence of advanced fibrosis/cirrhosis in people with hemophilia: results from a non-invasive screening based on international hepatological recommendations.

Sunday, June 23, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Vincenzo La Mura, MD PhD

Associate Professor
Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
Milan, Lombardia, Italy

Co-Author(s)

NB

Niccolò Bitto

Clinician
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi and Thrombosis center, Milan, Italy
Milan, Lombardia, Italy
CC

Camilla Caputo

Resident
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi and Thrombosis center, Milan, Italy
Milan, Lombardia, Italy
SS

Simona Maria Siboni

Physician
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center
Milan, Lombardia, Italy
Sara Arcudi, MD, PhD

Clinician
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Alessandro Ciavarella

Clinician
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Roberta Gualtierotti, PhD

Associate Professor
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy
Milan, Lombardia, Italy
GO

Giovanna Oberti

Clinician
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Unit of Internal Medicine and Metabolic Diseases, Milan, Italy
Milan, Lombardia, Italy
GP

Giuseppina Pisano

Clinician
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Unit of Internal Medicine and Metabolic Diseases, Milan, Italy
Milan, Lombardia, Italy
AF

Anna Ludovica Fracanzani

Director
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Unit of Internal Medicine and Metabolic Diseases, Milan, Italy;
Milan, Lombardia, Italy
AS

Angelo Sangiovanni

Clinician
Foundation I.R.C.C.S. Ca’ Granda, Ospedale Maggiore Policlinico, Gastroenterology and Hepatology, Milan, Italy.
Milan, Lombardia, Italy
Flora Peyvandi, MD, PhD

Full Professor
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milan, Lombardia, Italy

Background: People with hemophilia (PWH) are at risk of advanced fibrosis/cirrhosis due to the high prevalence of HCV/HBV exposure and steatotic liver disease (SLD). Liver stiffness measurement (LSM) by Fibroscan is the most recommended tool to rule out advanced fibrosis/cirrhosis in candidates to gene therapy for hemophilia A and B, but it may be inaccurate after HCV clearance, whilst a hepatological evaluation may reduce this misclassification. Conversely, in non-virus exposed PWH, the use of FIB-4 formula (e.g. age, transaminases, platelet count) could be an useful screening tool to individuate patients deserving LSM and further hepatological evaluation.

Aims: We conducted a prospective study to explore the prevalence of advanced fibrosis/cirrhosis in a large consecutive series of PWH.

Methods: Virus exposed subjects were addressed to liver ultrasound (US), LSM and an accurate check of risk factors of chronic liver damage. Non-virus exposed subjects were first screened by FIB-4, reserving instrumental US+LS and hepatological evaluation for PWH with FIB-4 over the recommended threshold of risk of 1.3 points.

Results: 395 subjects were included. Among the 165 virus exposed subjects, 4 (2.4%) did not present any risk factor of chronic liver damage and were discharged from periodical hepatological evaluation. One-hundred and eight (65.5%) presented at least one active risk factor of chronic liver damage and 53(32.1%) advanced fibrosis/cirrhosis. Among the 230 non-virus exposed subjects, just 8 (3.5%) had a FIB-4>1.3. Four subjects (50%) refused the evaluation, 3(37.5%) were discharged, and 1(12.5%) maintained follow-up due to at least one active risk factor of chronic liver damage.

Conclusion(s): PWH previously exposed to HCV/HBV infection remain at risk of advanced fibrosis/cirrhosis and need hepatological evaluation. The screening algorithm based on FIB-4 and LS evaluation may not meet patient acceptance among non-virus exposed subjects. However, this subgroup seems at very low prevalence of advanced fibrosis/cirrhosis.