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Blood, Vol. 92 No. 9 (November 1), 1998:
pp. 3460-3464
A Multicenter Prospective Study on the Risk of Acquiring Liver
Disease in Anti-Hepatitis C Virus Negative Patients Affected
From Homozygous  -Thalassemia
Daniele Prati,
Alberto Zanella,
Elena Farma,
Claudia De Mattei,
Patrizia Bosoni,
Manuela Zappa,
Alessandra Picone,
Fulvio Mozzi,
Paolo Rebulla,
Maria Domenica Cappellini,
Jean-Pierre Allain, and
Girolamo Sirchia for the Cooleycare Cooperative Group
From the Centro Trasfusionale e di Immunologia dei Trapianti,
Servizio Autonomo per il Prelievo e la Conservazione di Organi e
Tessuti, Divisione di Ematologia, and Centro Anemie Congenite, IRCCS
Ospedale Maggiore di Milano, Milano, Italy; and the Division of
Transfusion Medicine, University of Cambridge, Cambridge, UK.
Although the risk of transfusion-transmitted hepatitis has been
recently reduced, transfusion-dependent  -thalassemia patients may
still develop liver disease due to viral infection or iron overload. We
assessed the frequency and causes of liver dysfunction in a cohort of
anti-hepatitis C virus (HCV) negative thalassemics. Of 1,481 thalassemics enrolled in 31 centers, 219 (14.8%) tested anti-HCV by second-generation assays; 181 completed a
3-year follow-up program consisting of alanine-aminotransferase (ALT)
measurement at each transfusion and anti-HCV determination by
third-generation enzyme-immunoassay (EIA-3) at the end of study. Serum
ferritin levels were determined at baseline and at the end of
follow-up. Ten patients were anti-HCV+ by EIA-3 at the
end of follow-up. Of them, seven were already positive in 1992 to 1993 when the initial sera were retested by EIA-3, one tested indeterminate
by confirmatory assay, and two had true seroconversion (incidence,
4.27/1,000 person years; risk of infection, 1/7,100 blood units, 95%
confidence interval [CI], 1 in 2,000-1 in 71,000 units). At baseline,
67 of 174 thalassemics had abnormal ALT. Of those with normal ALT,
seven subsequently developed at least one episode of moderate ALT
increase (incidence, 24.6/1,000 person-years). All of the 20 patients
with ferritin values  3,000 ng/mL had clinically relevant ALT
abnormalities, as compared with 53 of 151 with <3,000 ng/mL (P
< .005). Hepatic dysfunction is still frequent in thalassemics.
Although it is mainly attributable to siderosis and primary HCV
infection, the role of undiscovered transmissible agents cannot be
excluded.
© 1998 by The American Society of Hematology.
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