-Author name in bold denotes the presenting author
-Asterisk * with author name denotes a Non-ASH member
Clinically Relevant Abstract denotes an abstract that is clinically relevant.

PhD Trainee denotes that this is a recommended PHD Trainee Session.

Ticketed Session denotes that this is a ticketed session.

3783 Liver Health in Patients with Hemophilia: Residual Risk Factors of Liver-Related Complications after HCV ClearanceClinically Relevant Abstract

Program: Oral and Poster Abstracts
Session: 322. Disorders of Coagulation or Fibrinolysis: Clinical and Epidemiological: Poster III
Hematology Disease Topics & Pathways:
Bleeding and Clotting, Clinical Practice (Health Services and Quality), hemophilia, Diseases, Adverse Events
Monday, December 12, 2022, 6:00 PM-8:00 PM

Vincenzo La Mura, MD, PhD1,2*, Niccolò Bitto, MD2*, Cecilia Capelli, MD1*, Simona Maria Siboni, MD2*, Sara Arcudi, MD1,2*, Alessandro Ciavarella, MD2,3*, Roberta Gualtierotti4,5* and Flora Peyvandi, MD1,6

1Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
2Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
3Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy
4Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
5Department of Pathophysiology and Transplantation, Università Degli Studi Di Milano, Milan, Italy
6Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy

Background and Aim: Hepatitis C virus (HCV) is highly prevalent in patients with hemophilia, but today almost all patients achieve a sustained virological response (SVR) to therapy. Unfortunately, other risk factors (e.g. metabolic and/or alcohol) can make chronic hepatitis continue even after SVR and, potentially, evolve to cirrhosis. We herein report data from a prospective hepatological screening program aimed at evaluating any relevant risk factor of liver-related complications after SVR in patients with hemophilia.

Methods: We enrolled 75 HCV-patients (median age: 54; range: 36-80): 68/7 hemophilia A/B, 63% suffering from a severe inherited bleeding disorder. At time of inclusion, 22 patients (29%) had achieved a SVR after successful interferon-based therapies, 48 (64%) after third generation antiviral agents, 5 (7%) had obtained a spontaneous virus clearance. All patients were screened for active etiologic factors of chronic hepatitis, biochemistry, liver stiffness measurement (LSM) and ultrasound-exploration (US) to rule in/out cirrhosis. Invasive diagnostic tests were adopted when appropriate. Data before SVR were available in 44 patients for pre/post-SVR analysis to explore their potential diagnostic accuracy to rule out an advanced chronic liver disease at time of the screening.

Results: Thirty-one patients (41%) presented at least one active risk factor of potential chronic liver damage, metabolic syndrome being the most prevalent (36%). Twenty-nine patients (39%) had also HIV/HBV infection although adequately treated at time of the screening. Steatosis was detected by US in 37 patients (48%). Based on clinical, biochemical and/or morphological criteria 21 patients (28%) had signs cirrhosis. Among them, 4 patients (5%) had esophageal varices needing treatment, 3 (4%) hepatocellular carcinoma (HCC). One HCC was detected in a non-cirrhotic liver but was associated with metabolic comorbidities, 2 were HCC out of transplantation criteria and were addressed to a multi-disciplinary down-staging approach (e.g. surgery, chemoembolization, chemotherapy). Finally 2 of HCC were transplanted. All the non-invasive tests for cirrhosis (e.g. LSM, APRI, Fib4, Forns score) were sensibly reduced after virus clearance (p<0.05). On the contrary typical US-signs of cirrhosis did not significantly change after SVR. Eleven patients had LSM between 6-9 KPa. Interestingly, 3 of them had irregular liver surface, 1 caudate hypertrophic liver lobe, 6 splenomegaly, suggesting that non-invasive tests may not accurately rule out an advanced chronic liver disease after HCV clearance even for low levels of LSM.

Conclusions: A residual risk of chronic liver damage after HCV-clearance is frequently observed. Non-invasive tests of fibrosis dramatically change after SVR and could be inaccurate to rule out cirrhosis. A specific diagnostic work-up is mandatory to preserve liver-health in this clinical setting.

Disclosures: La Mura: Gore: Speakers Bureau; Alfasigma: Speakers Bureau; Sanofi: Other: Travel grant; Biomarin: Other: Travel grant; Takeda: Other: Travel Grant; CSL Behring: Speakers Bureau. Ciavarella: Bayer: Consultancy, Other: Bayer Hemophilia Awards Fellowship. Gualtierotti: Takeda: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Bayer: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Pfiser: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novo Nordisk: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Roche: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Peyvandi: Roche: Honoraria; Takeda: Honoraria; BioMarin: Honoraria; Grifols: Honoraria; Sobi: Honoraria; Sanofi: Honoraria.

*signifies non-member of ASH