Haematologica
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Published online 12 June 2008
Haematologica, Vol 93, Issue 8, 1243-1246 doi:10.3324/haematol.12554
Copyright © 2008 by Ferrata Storti Foundation
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Thalassemia Syndromes

Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C

Vito Di Marco1, Marcello Capra2, Francesco Gagliardotto2, Zelia Borsellino2, Daniela Cabibi3, Francesco Barbaria1, Donatella Ferraro4, Liana Cuccia2, Giovanni Battista Ruffo2, Fabrizio Bronte1, Rosa Di Stefano4, Piero L. Almasio1, Antonio Craxì1

1 Unità Complessa di Gastroenterologia ed Epatologia, Dipartimento Biomedico di Medicina Interna e Specialistica (Di.Bi.MI.S.), University of Palermo
2 Unità Complessa Ematologia-Emoglobinopatie, Ospedale Pediatrico "G. Di University of Palermo, Italy Cristina", ARNAS Civico, Palermo
3 Servizio di Istologia Patologica, University of Palermo
4 Servizio di Virologia

Correspondence: Vito Di Marco, Cattedra di Gastroenterologia, Dipartimento Biomedico di Medicina Interna e Specialistica, (DiBiMIS), University of Palermo, Piazza delle Cliniche 2, 90127 Palermo, Italy. E-mail:vito.dimarco{at}tin.it

Iron overload and hepatitis virus C infection cause liver fibrosis in thalassemics. In a monocentric retrospective analysis of liver disease in a cohort of 191 transfusion-dependent thalassemics, in 126 patients who had undergone liver biopsy (mean age 17.2 years; 58 hepatitis virus C-RNA positive and 68 hepatitis virus C-RNA negative) the liver iron concentration (median 2.4 mg/gr dry liver weight) was closely related to serum ferritin levels (R = 0.58; p<0.0001). Male gender (OR 4.12) and serum hepatitis virus C-RNA positivity (OR 11.04) were independent risk factors for advanced liver fibrosis. The majority of hepatitis virus C-RNA negative patients with low iron load did not develop liver fibrosis, while hepatitis virus C-RNA positive patients infected with genotype 1 or 4 and iron overload more frequently developed advanced fibrosis. Hepatitis virus C infection is the main risk factor for liver fibrosis in transfusion-dependent thalassemics. Adequate chelation therapy usually prevents the development of liver fibrosis in thalassemics free of hepatitis virus C-infection and reduces the risk of developing severe fibrosis in thalassemics with chronic hepatitis C.

Key words: thalassemia, liver iron concentration, ferritin, LIC, hepatitis C virus, serum HCV RNA, liver fibrosis, cirrhosis.


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Treatment of hepatitis C in patients with thalassemia
Emanuele Angelucci, Federica Pilo
Haematologica 2008 93: 1121-1123. [Full Text] [PDF]



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E. Angelucci and F. Pilo
Treatment of hepatitis C in patients with thalassemia
Haematologica, August 1, 2008; 93(8): 1121 - 1123.
[Full Text] [PDF]


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