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Prepublished online as a Blood First Edition Paper on May 17, 2002; DOI 10.1182/blood-2002-01-0306. This Article Full Text (PDF) Erratum (v101,p2460) All Versions of this Article: 2002-01-0306v1 100/5/1566    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Wanless, I. R Articles by Cohen, A. R Search for Related Content PubMed PubMed Citation Articles by Wanless, I. R Articles by Cohen, A. R Related Collections Related Letter in Blood Online Social Bookmarking                   What's this? Submitted January 30, 2002 Accepted April 11, 2002 LACK OF PROGRESSIVE HEPATIC FIBROSIS DURING LONG TERM THERAPY WITH DEFERIPRONE IN SUBJECTS WITH TRANSFUSION-DEPENDENT BETA-THALASSEMIA Ian R Wanless*, George Sweeney, Amar P Dhillon, Maria Guido, Antonio Piga, Renzo Galanello, M. Rita Gamberini, Elias Schwartz, and Alan R Cohen Department of Laboratory Medicine and Pathobiology, Toronto General Hospital, Toronto, Ontario, Canada Department of Medicine, McMaster University, Hamilton, Ontario, Canada Department of Histopathology, Royal Free and University College Medical School, London, United Kingdom Dipartimento di Anatomia Patologica, Universita degli Studi di Padova, Padova, Italy Department of Pediatrics, Universita degli Studi di Torino, Torino, Italy Instituto di Clinica e Biologia Dell'Eta'Evolutiva, Cagliari, Italy Divisione Pediatrica, Arcispedale S. Anna - Azienda Ospedaliera di Ferrara, Ferrarra, Italy Department of Pediatrics, Jefferson Medical College, Philadelphia, PA, USA Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA * Corresponding author; email: ian.wanless{at}utoronto.ca. Patients with thalassemia major require life-long chelation therapy to prevent iron-induced organ damage. The orally active chelator deferiprone has been proposed as an alternative for patients unable or unwilling to use deferoxamine. One report has concluded that deferiprone may worsen hepatic fibrosis in patients with thalassemia, while others have found no detrimental effect. A panel of 3 pathologists evaluated 112 coded liver biopsies obtained from 56 patients before and after deferiprone therapy. Fibrosis was scored with the Laennec and Ishak systems. The mean interval between liver biopsies was 3.1 years (range 1.2-4.9). In 11 patients seronegative for hepatitis C, fibrosis scores before and after therapy were 1.12±1.07 and 0.97±0.84 (P=0.42) using the Ishak system, and 0.71±0.65 and 0.70±0.53 (P=0.91) using the Laennec system. Among 45 patients seropositive for hepatitis C, fibrosis scores before and after therapy were 1.91±1.13 and 2.04±1.30 (P=0.43) using the Ishak system and 1.26±0.73 and 1.35±0.90 (P=0.41) using the Laennec system. When the data set was limited to biopsies that each contained 6 portal tracts (31 patients), analysis still showed no significant change in fibrosis with time. Using the Laennec system, the fibrosis score increased by >1 level in no patients without hepatitis C and in 1 patient with hepatitis C, and decreased by >1 level in none of the 56 patients. This analysis of the largest collection of liver biopsies reported to date in patients receiving deferiprone demonstrates no evidence of deferiprone-induced progression of hepatic fibrosis during long-term therapy. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Letter in Blood Online: Deferiprone and hepatic fibrosis Gary M. Brittenham, David G. Nathan, Nancy F. Olivieri, John B. Porter, Martin Pippard, Elliott P. Vichinsky, David J. Weatherall, Ian R. Wanless, George Sweeney, Amar P. Dhillon, Maria Guido, Antonio Piga, Renzo Galanello, Elias Schwartz, and Alan Cohen Blood 2003 101: 5089-5091. [Full Text] [PDF] This article has been cited by other articles: M. Thursz Iron, haemochromatosis and thalassaemia as risk factors for fibrosis in hepatitis C virus infection Gut, May 1, 2007; 56(5): 613 - 614. [Full Text] [PDF] M.A. Tanner, R. Galanello, C. Dessi, G.C. Smith, M.A. Westwood, A. Agus, M. Roughton, R. Assomull, S.V. Nair, J.M. Walker, et al. 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Vichinsky, D. J. Weatherall, I. R. Wanless, G. Sweeney, A. P. Dhillon, et al. Deferiprone and hepatic fibrosis Blood, June 15, 2003; 101(12): 5089 - 5091. [Full Text] [PDF] G. Constantinou, S. Melides, B. Modell, M. Spino, F. Tricta, D. G. Nathan, and D. J. Weatherall The Olivieri Case N. Engl. J. Med., February 27, 2003; 348(9): 860 - 863. [Full Text] [PDF] E. Beutler, A. V. Hoffbrand, and J. D. Cook Iron Deficiency and Overload Hematology, January 1, 2003; 2003(1): 40 - 61. [Abstract] [Full Text] [PDF]

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