Medicina Interna 1, Azienda di Rilievo Nazionale ad Alta Specializzazione Civico - Di Cristina - Benfratelli, Piazzale Leotta 4, 90100, Palermo, Italy.
BMC Gastroenterol. 2020 Dec 9;20(1):409. doi: 10.1186/s12876-020-01542-2.
Hepatocellular carcinoma as a complication is linked to improved outcomes of thalassemia.
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.
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.
肝癌作为并发症与地中海贫血症的改善结果相关。
已发表的数据表明地中海贫血症患者中 HCC 的发病率约为 2%。然而，由于地中海贫血症在地中海贫血症高发的许多欠发达国家中较为普遍，这些国家的患者可能尚未进行 HCC 筛查，因此该疾病的负担可能更高。通过输血预防 HCV 感染、HCV 治疗和充分的铁螯合等都是预防地中海贫血症患者 HCC 的手段。在存在风险因素的情况下，对地中海贫血症患者进行 HCC 筛查似乎是合理的。地中海贫血症患者 HCC 的管理不应与非地中海贫血症患者有所不同。然而，由于存在相关合并症，肝移植可能具有挑战性，应仅保留给高度选择的病例。地中海贫血症患者 HCC 的管理决策应遵循多学科努力。此外，由于关于该问题的已发表数据较少，未来的多中心国际研究将有所帮助。
在 BMC 胃肠病学中，意大利专家组报告了地中海贫血症 HCC 管理指南的一项值得称赞的努力的结果。然而，由于关于该主题的已发表数据较少，因此一些结论存在灰色地带，是争议的原因。
