Time to define a new strategy for management of hepatocellular carcinoma in thalassaemia?

We have read with interest the article entitled 'Hepatocellular carcinoma in thalassaemia. An update of the Italian registry' (Borgna-Pignatti et al, 2014). The paper witnesses a spread of interest on the issue. The Authors reported 62 new cases of hepatocellular carcinoma (HCC) among 5·855 thalassaemic patients (cumulative incidence 1%) in the period between 2002 and December 2012. The paper enlightens on the real weight of the issue, demonstrating that HCC represents a substantial complication in thalassaemia. In fact, the same authors had previously published the results of a survey of data collected from the entire population of Italian patients with thalassaemia, reporting only 22 cases of HCC in that retrospective study 10 years ago (Borgna-Pignatti et al, 2004a). Given that survival in thalassaemia recently has shown a dramatic improvement as a consequence of improved transfusion regimens and regular chelation (Borgna-Pignatti et al, 2004b; Ladis et al, 2011), the magnitude of the neoplastic issue is probably the expression of a real increase in HCC incidence in thalassaemia, rather than only an increased awareness of the problem. The incidence of HCC in thalassaemia was reported in 2% of the patients screened with ultrasound in a 1-year prospective study (Mancuso et al, 2006), as compared with 1–8% per year in the population of non-thalassaemic cirrhotic patients (Ioannou et al, 2007). However, the age at diagnosis of HCC in thalassaemic patients is younger than that reported in patients without thalassaemia, suggesting that the concomitance of different risk factors, namely haemochromatosis and chronic viral hepatitis, could enhance the risk of HCC development. The few data published would suggest the utility of screening for HCC detection in thalassaemia, although there is no evidence of a survival benefit due to the rarity of this haematological condition. The management of HCC in thalassaemia should probably follow the same indications recommended for HCC in non-thalassaemic patients (European Association for the Study of the Liver; European Organisation for Research & Treatment of Cancer, 2012). Published data report encouraging results of surgical and loco-regional treatments (Mancuso et al, 2005; Mancuso, 2010). Only a few thalassaemic patients have undergone liver transplantation. The reason for this is probably partly due to comorbidities, mainly cardiac, and partly to the fact that thalassaemia per se is generally considered a contraindication for liver transplantation. In their recent article, Borgna-Pignatti et al (2014) reported that two of the three transplanted patients died after liver transplantation for reasons unrelated to thalassaemia. In conclusion, HCC is an emerging new issue in thalassaemia. Ultrasound screening should be offered to thalassaemic patients, especially for those with one or more risk factors for HCC (i.e. iron overload, hepatitis C virus infection, hepatitis B virus infection, advanced liver fibrosis or cirrhosis). Multidisciplinary management should be implemented. Finally, liver transplantation could represent a reliable option to cure in highly selected patients. None to declare. None to declare. Andrea Mancuso conceived, wrote and revised the article. Giovanni Perricone contribuited to article writing. All authors approved the final version of the manuscript.