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Ann Hematol ; 92(6): 739-46, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23412560

RESUMO

Chronic hepatitis C (CHC) and iron overload are the main causes of liver disease in ß-thalassemia major (ßTM). There is limited data regarding the course of CHC in this population. All patients (n=144) from the thalassemia centre of the University Hospital of Patras were evaluated (January 1981 to June 2012). Patients were classified into group A (n=57), which consisted of patients with CHC, who either had received antiviral treatment (n=49) or not (n=8), and group B which included 87 patients without CHC. Nineteen patients died during follow-up (median: 257.5 months (1-355)). Survival rates were 84.2 % and 88.5 % for group A and B, respectively. The causes of death were heart failure (63.2 %), accident (10.5 %), sepsis (5.3 %), liver failure (5.3 %), hepatocellular carcinoma (HCC) (5.3 %), non-Hodgkin lymphoma (5.3 %) and multiorgan failure (5.3 %). There were no differences in total survival between the two groups (p=0.524). In the multivariate analysis, survival was neither correlated with CHC (p=ns), nor with anti-HCV treatment (p=ns), whereas independent negative predictors were presence of heart failure (p<0.001), presence of malignancy other than HCC (p=0.001) and non-adherence to chelation treatment (p=0.013). Predictive factors for the development of cirrhosis were: CHC (p<0.001), age>35 years (p=0.007), siderosis grade 3/4 (p=0.029) and splenectomy (p=0.001); however, multivariately, only siderosis grade 3/4 was found to be significant (p=0.049). In this study, survival of patients with ßTM was mainly associated with heart failure, presence of malignancy other than HCC and non-adherence to chelation treatment, rather than with liver disease. Multicentre studies need to be designed to define more accurately the indications of antiviral treatment in this population.


Assuntos
Hepatite C Crônica/epidemiologia , Talassemia beta/epidemiologia , Adolescente , Adulto , Antivirais/uso terapêutico , Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/etiologia , Causas de Morte , Terapia por Quelação , Criança , Pré-Escolar , Comorbidade , Feminino , Grécia/epidemiologia , Hepatite C Crônica/tratamento farmacológico , Hepatite C Crônica/etiologia , Hepatite C Crônica/patologia , Humanos , Lactente , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/epidemiologia , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/patologia , Sobrecarga de Ferro/terapia , Estimativa de Kaplan-Meier , Fígado/química , Fígado/patologia , Fígado/virologia , Cirrose Hepática/epidemiologia , Cirrose Hepática/etiologia , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/etiologia , Masculino , Adesão à Medicação , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Esplenectomia/estatística & dados numéricos , Reação Transfusional , Adulto Jovem , Talassemia beta/terapia
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