Italian Society for the Study of Thalassemia and Haemoglobinopathies, Rome, Italy.
Blood. 2010 Oct 21;116(16):2875-83. doi: 10.1182/blood-2009-11-248724. Epub 2010 Jun 15.
Chelation therapy with new drugs prevents cardiac damage and improves the survival of thalassemia patients. Liver diseases have emerged as a critical clinical issue. Chronic liver diseases play an important role in the prognosis of thalassemia patients because of the high frequency of viral infections and important role of the liver in regulating iron metabolism. Accurate assessment of liver iron overload is required to tailor iron chelation therapy. The diagnosis of hepatitis B virus- or hepatitis C virus-related chronic hepatitis is required to detect patients who have a high risk of developing liver complications and who may benefit by antiviral therapy. Moreover, clinical management of chronic liver disease in thalassemia patients is a team management issue requiring a multidisciplinary approach. The purposes of this paper are to summarize the knowledge on 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 in thalassemia patients with chronic hepatitis B or C virus or cirrhosis.
螯合疗法联合新药可预防心脏损伤,提高地中海贫血患者的生存率。肝脏疾病已成为一个关键的临床问题。慢性肝脏疾病在地中海贫血患者的预后中起着重要作用,因为病毒感染的频率高,肝脏在调节铁代谢方面也起着重要作用。需要准确评估肝脏铁过载,以调整铁螯合疗法。需要诊断乙型肝炎病毒或丙型肝炎病毒相关的慢性肝炎,以发现发生肝脏并发症风险高且可能受益于抗病毒治疗的患者。此外,地中海贫血患者慢性肝脏疾病的临床管理是一个团队管理问题,需要多学科方法。本文的目的是总结病毒感染的流行病学和传播风险的知识,分析诊断慢性肝脏疾病的有创和无创方法,报告慢性病毒性肝炎的临床病程知识,并提出乙型肝炎病毒或丙型肝炎病毒或肝硬化的地中海贫血患者的抗病毒治疗管理建议。