Abstract
Background and Objectives. Updated information on liver disease in transfusion-dependent β-thalassemia is lacking. We conducted a multicenter study within the Cooleycare Group to describe the clinical and histopathological features of liver disease in currently treated thalassemics. Design and Methods. Two-hundred and three thalassemics with laboratory signs of liver disease were eligible. Liver biopsy was performed in the 129 (63.5%) who consented (age 26±7 years). Biological samples were sent to the central laboratory. Results. Anti-hepatitis C virus (HCV) antibodies were found in 118 patients (91%), 85 (72%) of whom were viremic. Ninety-one patients (70%) had abnormal aminotransferase concentrations. In the 117 liver biopsies that met the criteria for evaluation (88%), the median Ishak's necroinflammatory and fibrosis scores were 4 (range, 0-9) and 2 (range, 0-6), respectively. Significant fibrosis (score ≥3) was found in 53 (45%); 9 (8%) had cirrhosis. At multivariate analysis, necroinflammation was related to HCV viremia, and fibrosis to increased serum aminotransferases, higher iron stores (including serum ferritin, Deugnier's total iron score, and liver iron content) and male gender (p
| Original language | English |
|---|---|
| Pages (from-to) | 1179-1186 |
| Number of pages | 8 |
| Journal | Haematologica |
| Volume | 89 |
| Issue number | 10 |
| Publication status | Published - Oct 2004 |
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Keywords
- Blood transfusion
- Hepatitis C
- Iron overload
- Liver disease
- Thalassemia
ASJC Scopus subject areas
- Hematology
Cite this
Clinical and histological characterization of liver disease in patients with transfusion-dependent β-thalassemia. A multicenter study of 117 cases. / Prati, Daniele; Maggioni, Marco; Milani, Silvano; Cerino, Mario; Cianciulli, Paolo; Coggi, Guido; Forni, Gian Luca; Magnano, Carmelo; Meo, Anna; Gramignoli, Roberto; Rebulla, Paolo; Fiorelli, Gemino; Cappellini, Maria Domenica.
In: Haematologica, Vol. 89, No. 10, 10.2004, p. 1179-1186.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Clinical and histological characterization of liver disease in patients with transfusion-dependent β-thalassemia. A multicenter study of 117 cases
AU - Prati, Daniele
AU - Maggioni, Marco
AU - Milani, Silvano
AU - Cerino, Mario
AU - Cianciulli, Paolo
AU - Coggi, Guido
AU - Forni, Gian Luca
AU - Magnano, Carmelo
AU - Meo, Anna
AU - Gramignoli, Roberto
AU - Rebulla, Paolo
AU - Fiorelli, Gemino
AU - Cappellini, Maria Domenica
PY - 2004/10
Y1 - 2004/10
N2 - Background and Objectives. Updated information on liver disease in transfusion-dependent β-thalassemia is lacking. We conducted a multicenter study within the Cooleycare Group to describe the clinical and histopathological features of liver disease in currently treated thalassemics. Design and Methods. Two-hundred and three thalassemics with laboratory signs of liver disease were eligible. Liver biopsy was performed in the 129 (63.5%) who consented (age 26±7 years). Biological samples were sent to the central laboratory. Results. Anti-hepatitis C virus (HCV) antibodies were found in 118 patients (91%), 85 (72%) of whom were viremic. Ninety-one patients (70%) had abnormal aminotransferase concentrations. In the 117 liver biopsies that met the criteria for evaluation (88%), the median Ishak's necroinflammatory and fibrosis scores were 4 (range, 0-9) and 2 (range, 0-6), respectively. Significant fibrosis (score ≥3) was found in 53 (45%); 9 (8%) had cirrhosis. At multivariate analysis, necroinflammation was related to HCV viremia, and fibrosis to increased serum aminotransferases, higher iron stores (including serum ferritin, Deugnier's total iron score, and liver iron content) and male gender (p
AB - Background and Objectives. Updated information on liver disease in transfusion-dependent β-thalassemia is lacking. We conducted a multicenter study within the Cooleycare Group to describe the clinical and histopathological features of liver disease in currently treated thalassemics. Design and Methods. Two-hundred and three thalassemics with laboratory signs of liver disease were eligible. Liver biopsy was performed in the 129 (63.5%) who consented (age 26±7 years). Biological samples were sent to the central laboratory. Results. Anti-hepatitis C virus (HCV) antibodies were found in 118 patients (91%), 85 (72%) of whom were viremic. Ninety-one patients (70%) had abnormal aminotransferase concentrations. In the 117 liver biopsies that met the criteria for evaluation (88%), the median Ishak's necroinflammatory and fibrosis scores were 4 (range, 0-9) and 2 (range, 0-6), respectively. Significant fibrosis (score ≥3) was found in 53 (45%); 9 (8%) had cirrhosis. At multivariate analysis, necroinflammation was related to HCV viremia, and fibrosis to increased serum aminotransferases, higher iron stores (including serum ferritin, Deugnier's total iron score, and liver iron content) and male gender (p
KW - Blood transfusion
KW - Hepatitis C
KW - Iron overload
KW - Liver disease
KW - Thalassemia
UR - http://www.scopus.com/inward/record.url?scp=6344265915&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=6344265915&partnerID=8YFLogxK
M3 - Article
C2 - 15477201
AN - SCOPUS:6344265915
VL - 89
SP - 1179
EP - 1186
JO - Haematologica
JF - Haematologica
SN - 0390-6078
IS - 10
ER -