Hepatitis C Virus Infection in Patients With Hemoglobinopathies
Hepatitis C Virus Infection in Patients With Hemoglobinopathies
Progression of liver fibrosis in patients with hemoglobinopathies is strongly related to the
 severity of iron overload and the presence of chronic hepatitis C virus (HCV) infection.
 Effective iron chelation therapy and HCV infection eradication are efficacy to prevent liver
 complications. EASL and AASLD guidelines recommend interferon-free regimens for the treatment
 of HCV infection in patients with hemoglobinopathies. However, data regarding the use of
 direct-acting antiviral drugs (DAAs) in this patient population are very few This large,
 observational study evaluated the safety and efficacy of standard therapy with DAAs in a
 large Italian cohort of with hemoglobinopathies, chronic HCV infection and advanced liver
 fibrosis.
Many patients with hemoglobinopathies have been infected with hepatitis C virus (HCV) through
 blood transfusion, mostly before screening of blood donors was introduced in 1992. The
 reported prevalence of anti-HCV-positive thalassemia patients varies between 4.4% in Turkey
 and 85.4% in Italy. HCV infection is associated with decompensated cirrhosis, hepatocellular
 carcinoma and other liver complications, especially if left untreated. Prevalence of
 cirrhosis in thalassemia patients up to 32% have been reported.

 Thalassemia patients with cirrhosis have an increased risk of death. Progression of liver
 fibrosis is strongly related to the presence of chronic HCV infection and extent of iron
 overload. Thalassemia patients with elevated serum aminotransferase levels for >6 months
 should be tested for HCV infection and, in the event of HCV infection, HCV genotyping is
 recommended in order to plan antiviral therapy and the likelihood of response. Noninvasive
 transient elastography (FibroScan®) can be used to determine the presence of fibrosis in
 thalassemia patients with HCV infection.

 Effective chelation therapy and treatment of HCV infection are needed in order to prevent
 liver complications and decrease morbidity and mortality. For many years, pegylated
 interferon (peg-IFN) plus ribavirin was the standard of care for the treatment of chronic HCV
 infection and decompensated cirrhosis. Studies of peg-IFN plus ribavirin have demonstrated
 sustained virological response (SVR) rates of 25-64% in patients with thalassemia and HCV
 infection. However, peg-IFN and ribavirin are both associated with anemia.
 Ribavirin-associated hemolysis leads to an increased requirement for blood transfusions,
 which in turn can lead to worsening of iron overload. Therefore, European Association for the
 Study of the Liver (EASL) 2015 guidelines recommend interferon-free regimens for the
 treatment of HCV infection in patients with hemoglobinopathies. The aim of this study was to
 evaluate the safety and efficacy of DAA standard regimens in patients with hemoglobinopathies
 and chronic HCV liver disease treated in Italy.

 Antiviral treatments were administered according to Italian Medicines Agency (AIFA)
 guidelines.
Inclusion Criteria:

 -  Patients with hemoglobinopathies, chronic hepatitis due to HCV and the presence of
 fibrosis (defined as Fibroscan® stiffness ≥10 kPa) or a bioptic evaluation of
 cirrhosis (same ISHAK fibrosis score) determined within 6 months previously. Patients
 with extrahepatic manifestations of chronic hepatitis C virus infection
 (cryoglobulinemia with organ damage, B-lymphoproliferative disorders) were also
 included.

 Exclusion Criteria:

 -  Patients with active cancer, including hepatocellular carcinoma, and pregnant or
 lactating females were excluded from the study