title: Liver iron and fibrosis during long-term treatment with deferiprone in Swiss thalassaemic patients.
abstract: Serum ferritin levels, hepatic histology and iron concentration were studied in a 'veteran' group of seven Swiss beta-thalassaemic patients after 93-99 months of treatment with the oral iron chelator deferiprone (L1), and another four patients who had received 54-82 months of L1 therapy. Despite continuous compliance, unexplained resurgence of serum ferritin levels occurred in 4/7 patients of the 'veteran' group after 4-5 years on L1. In three of these a concomitant increase of liver iron was also observed. Hepatic histology revealed significantly higher degrees of fibrosis in 6/11 hepatitis C (HC)-positive patients (fibrosis scores 1-5, mean 3.0) than in the HC-negative group (fibrosis score 0-2, mean 0.8). Two HC-negative patients had no detectable fibrosis after 98 and 93 months on deferiprone. Therefore the hepatic pathology in these patients cannot definitely be attributed as a side-effect of deferiprone. Chronic active hepatitis C and the accumulation of iron are the major causative factors to be considered.
authors.affiliation: Berne University Childrens' Hospital, Inselspital, Switzerland.
authors.forename: P
authors.initials: P
authors.lastname: Töndury
authors.forename: A
authors.initials: A
authors.lastname: Zimmermann
authors.forename: P
authors.initials: P
authors.lastname: Nielsen
authors.forename: A
authors.initials: A
authors.lastname: Hirt
mesh_terms: D000293:Adolescent; D000328:Adult; D002908:Chronic Disease; D000077543:Deferiprone; D005293:Ferritins / Q000097:blood*; D006526:Hepatitis C / Q000097:blood / Q000473:pathology; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008103:Liver Cirrhosis / Q000097:blood*; D008134:Long-Term Care; D011728:Pyridones / Q000627:therapeutic use*; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000473:pathology
chemical_list: D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D005293:Ferritins