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Prepublished online as a Blood First Edition Paper on May 17, 2002; DOI 10.1182/blood-2002-01-0306.
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Blood, 1 September 2002, Vol. 100, No. 5, pp. 1566-1569
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
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
From the Department of Laboratory Medicine and
Pathobiology, Toronto General Hospital and University of Toronto, ON,
Canada; McMaster University, Hamilton, ON, Canada; Department of
Histopathology, Royal Free and University College Medical School,
London, United Kingdom; Dipartimento di Anatomia Patologica and
Università degli Studi di Padova, Padua, Italy; Department of
Pediatrics, Università degli Studi di Torino, Turin, Italy;
Instituto di Clinica e Biologia Dell'Eta'Evolutiva, Cagliari, Italy;
Divisione Pediatrica, Arcispedale S Anna Azienda Ospedaliera di
Ferrara, Italy; and the Department of Pediatrics, Jefferson Medical
College, and the Department of Pediatrics, Children's Hospital of
Philadelphia, PA.
Patients with thalassemia major require lifelong 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,
whereas 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 years). In 11 patients seronegative for
hepatitis C, fibrosis scores before and after therapy were
1.12 ± 1.07 and 0.97 ± 0.84 (P = .42) with the use
of the Ishak system, and 0.71 ± 0.65 and 0.70 ± 0.53
(P = .91) with 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 = .43)
with the use of the Ishak system and 1.26 ± 0.73 and
1.35 ± 0.90 (P = .41) with the Laennec system. When
the data set was limited to biopsies that each contained 6 or more
portal tracts (31 patients), analysis still showed no significant
change in fibrosis with time. With the use of the Laennec system, the
fibrosis score did not increase by more than one level in any patients
without hepatitis C; it increased by more than one level in 1 patient
with hepatitis C; and it did not decrease by more than one level in any
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.
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