Evidence-based medicine and Management of Hepatocellular Carcinoma in Thalassemia
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COMMENTARY
Evidence-based medicine and Management of Hepatocellular Carcinoma in Thalassemia Andrea Mancuso*
Abstract Background: Hepatocellular carcinoma as a complication is linked to improved outcomes of thalassemia. Main body: Published data suggest an incidence of HCC in thalassemia of about 2%. However, since thalassemia is endemic in many under-developed countries where patients have not probably been screened for HCC yet, the burden of the disease could be higher. Prevention of HCV infection through blood transfusion, HCV treatment and adequate iron chelation are all tools to prevent HCC in thalassemia. In presence of risk factors, HCC screening seems appropriate for thalassemia. Management of HCC should not be different from that indicated for non thalassemics. However, liver transplantation can be challenging and should be reserved to highly selected cases, due to coexistence of relevant comorbidities. Decisions in the management of HCC in thalassemia should follow a multidisciplinary effort. Moreover, due to the paucity of published data about the issue, future multicenter international studies will be helpful. Short conclusion: In BMC Gastroenterology results of a commendable effort to guidelines for the management of HCC in thalassemia are reported by an Italian panel of experts. However, due to the paucity of published data about the topic, some conclusions rely on grey areas and are reason of debate. Keywords: Thalassemia, Cirrhosis, Hepatocellular carcinoma, Liver transplant Background Thalassaemias are rare inherited haemoglobin disorders, endemic in the Mediterranean Area and SouthEast Asia. Depending on severity, there are two clinical forms: thalassemia major (TM), in which severe anemia starts during the first year of life, requires life-long periodic transfusion management; and thalassemia intermedia (TI), in which anemia has later onset and is generally milder, allowing survival without any regular transfusion [1]. However, today the TM-TI dichotomy should be considered outdated, and, for both clinical and research purposes, thalassemias should be divided in transfusion This comment refers to the article available at https://doi.org/10.1186/s1287 6-020-01391-z. *Correspondence: [email protected] Medicina Interna 1, Azienda di Rilievo Nazionale ad Alta Specializzazione Civico - Di Cristina – Benfratelli, Piazzale Leotta 4, 90100 Palermo, Italy
dependent (TDT) and non-transfusion dependent (NTDT).
Main text Many thalassaemics are infected with either hepatitis C or B virus, due to blood transfusions, particularly those who were born before the 1990s. Moreover, most of the patients have haemochromatosis, which is the main cause of morbidity and mortality [1]. In the last decades, a sort of revolution has been happening in the management of thalassemia. In fact, whether previously thalassemia was a fatal disease in the early adulthood because of iron overload related heart failure, the outcome has recently improved due to the introduction of iron chelating drugs. Conseq
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