Haema 2011; 2(3): 282-288
by John Koskinas, Dimitrios Kountouras, Valia Fatourou
2nd Department of Internal Medicine, Medical School of Athens, Hippokration Hospital, Athens
The frequency of anti-HCV antibodies in patients with major beta-thalassemia is increased with age and ranges between 35-91% in adults with a middle age of 26yrs. HCV-RNA in serum is detected in 72% of anti-HCV(+) patients, with genotypes 1 and 4 over 60% of the total population, while 30% of them have normal aminotransferases. The pathogenesis and natural history of liver disease in thalassemia patients are related to the degree of liver hemosiderosis and chronic HCV infection. At the age of 26-35yrs, severe fibrosis (score >3) is observed in 45% of patients and cirrhosis in 8%. Furthermore, the significant prolongation of life expectancy in this group of patients during the last years has been unfortunately accompanied by an unexpected so far increase in the incidence of HCC. The group of patients that is principally affected is that of histological cirrhosis with or without HCV infection, with a median age of 40yrs. The decision to treat thalassemic patients for chronic HCV is strongly affected and repelled by the fact of multi-organ diseases, particularly cardiac. Monotherapy with classic or pegylated interferon-alpha achieves a sustained virological response (SVR) ranging between 13-40% while the addition of ribavirin increases it to 30-70%. The variation of these results relates to heterogeneity and small number of patients in the studies. Predictive parameters of non-response are older age, high liver iron, high serum HCV-RNA levels and advanced fibrosis. The most frequent reasons for treatment discontinuation are increased transfusion rate in the 48% of patients, neutropenia and thrombopenia. Favorable indications for treatment initiation are low LIC (<5 mg/gr) and absence of major cardiological complications. Addition of ribavirin to the treatment leads to significant improvement of SVR but it conveys an increase in transfusion rate. Although the new HCV protease inhibitors (boceprevir and telaprevir) are expected to increase the SVR in both naïve and non responders/relapsers genotype 1 patients they are associated with more side effects (haemolysis, skin rash) and therefore, they do not appear to be a promising option in the population of b-thalassemia.