RESUMO
AIM: We sought to evaluate the performance of transient elastography (TE) for the assessment of liver fibrosis in chronic hepatitis C (CHC) patients with beta-thalassemia. METHODS: Seventy-six CHC patients with beta-thalassemia underwent TE, liver biopsy, T2 -weighted magnetic resonance imaging (MRI) for the assessment of liver iron content (LIC) and laboratory evaluation. The accuracy of TE and its correlation with the other variables was assessed. RESULTS: TE values increased proportional to fibrosis stage (r = 0.404, P < 0.001), but was independent of T2 -weighted MRI-LIC (r = 0.064, P = 0.581). In multivariate analysis, fibrosis stage was still associated with the log-transformed TE score(standardized ß = 0.42 for F4 stage of METAVIR, P = 0.001). No correlation was noted between LIC and TE score (standardized ß = 0.064, P = 0.512). The area under the receiver operating characteristic curve for prediction of cirrhosis was 80% (95% confidence interval, 59-100%). A cut-off TE score of 11 had a sensitivity of 78% and specificity of 88.1% for diagnosing cirrhosis. The best cut-off values for "TE-FIB-4 cirrhosis score" comprising TE and FIB-4 and "TE-APRI cirrhosis score" combining TE with aspartate aminotransferase-to-platelet ratio index (APRI) both had 87.5% sensitivity and 91.04% specificity for the diagnosis of cirrhosis. CONCLUSION: Regardless of LIC, TE alone or when combined with FIB-4 or APRI, is a diagnostic tool with moderate to high accuracy to evaluate liver fibrosis in CHC patients with beta-thalassemia. However, because splenectomy in a proportion of our subjects might have affected the platelet count, the scores utilizing APRI and FIB-4 should be interpreted cautiously.
RESUMO
BACKGROUND: Beta thalassemia is one of the most common genetic disorders in the world. The aim of this study was to determine the frequency, characteristics, and pattern of malignancies in patients with beta thalassemia major (BTM) and beta thalassemia intermedia (BTI) in Iran. METHODS: We conducted a multicenter study via a retrospective chart review of patients with BTM and BTI between 2002 and 2007 in four thalassemia centers in Iran. A total of 4,630 records of patients with thalassemia were evaluated. Statistical analyses were done with SPSS software v. 15. P-values <0.05 were considered significant. RESULTS: We detected 11 patients with beta thalassemia who also had malignant disease. Five patients (45.4%) were diagnosed with lymphoma and five with leukemia. The proportion of patients with cancer was higher in those with BTI. Cancer was diagnosed in patients with thalassemia aged 0-39 years, but not in any of the older patients. In patients with thalassemia overall and in patients with BTI, the highest age-specific rate of cancer incidence was seen in children <10 years old, whereas in the BTM group the highest incidence was observed in patients 20-29 years old. There were no statistically significant differences between patients with BTM and BTI and cancer regarding age, sex, splenectomy, hemoglobin, ferritin level, or malignancy type. CONCLUSION: The coexistence of malignancy and beta thalassemia is not rare. Any alarming signs and symptoms including worsening anemia, splenomegaly, or lymphadenopathy should be a motive for concern because these symptoms may signal malignant disease.