IntroductionIn the last 4 decades, regular blood transfusions and chelation therapy have improved the survival of patients with thalassemia major. 1-3 Despite the progress on chelation therapy, cardiac complications remain the main cause of death among transfusiondependent thalassemia patients related to the susceptibility of cardiac cells to iron overload toxicity. 4,5 The interest in the clinical management of chronic liver diseases has been increasing, however, because of the high prevalence of viral infections in adult transfusion-dependent thalassemia patients and the central role of the liver in regulating the iron metabolism. 5,6 The assessment of heart and liver iron overload is required to tailor iron chelation therapy. Furthermore, the diagnosis of hepatitis B virus (HBV)-or hepatitis C virus (HCV)-related chronic hepatitis is required to identify patients who have a high risk of developing liver complications and who may obtain a benefit by antiviral therapy.The goals of this paper are to summarize the epidemiology and the risks of transmission of viral infections, to analyze invasive and noninvasive methods for the diagnosis of chronic liver disease, to report the knowledge on clinical course of chronic viral hepatitis, and to suggest the management of antiviral therapy. For personal use only. on May 12, 2018. by guest www.bloodjournal.org From An expert hepatologist prepared an initial draft based on systematic review of published literature by Medline search on viral hepatitis in thalassemia patients, examined recently published guidelines on the diagnosis, management, and treatment of chronic hepatitis B and chronic hepatitis C edited by the American Association for the Study of Liver Diseases, the European Association for the Study of the Liver, and the Asian-Pacific Association for the Study of the Liver 7-10 and the Consensus Development Conference sponsored by the National Institutes of Health. 11 Recommendations of the panel of experts were based insofar as possible on evidence from publications that reported data on thalassemia patients. If evidence from thalassemia patients was unavailable, the panel selected recommendations from published guidelines that were suitable for thalassemia patients. Recommendations were evaluated according to the Grading of Recommendations Assessment Development and Evaluation system and classified into 3 levels: high, moderate, or low 12 (Table 1). For each recommendation, the level of evidence (high, moderate, or low) and population studied (general population of patients with viral chronic hepatitis or thalassemia patients) was specified. The panel of experts discussed the draft issue by issue, and 3 experts on blood transfusion, iron metabolism, and viral hepatitis management reviewed the recommendations. Finally, 8 international external experts in pediatrics, hematology, and viral hepatitis reviewed and criticized the recommendations.
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What proportion of thalassemia patients have chronic viral infections?Worldwide, from 0.3% to 5.7% of thala...