Liver Unit, Department of Medical Sciences, Fondazione IRCCS "Casa Sollievo della Sofferenza", San Giovanni Rotondo, Italy.
Department of Radiological Sciences, Oncology and Pathology, "SAPIENZA" University of Rome; I.C.O.T. Hospital, Latina, Italy.
BMC Gastroenterol. 2020 Aug 3;20(1):251. doi: 10.1186/s12876-020-01391-z.
Beta-thalassemia represents a heterogeneous group of haemoglobin inherited disorders, among the most common genetic diseases in the world, frequent in the Mediterranean basin. As beta-thalassemia patients' survival has increased over time, previously unknown complications are observed with increasing frequency. Among them, an increased risk of hepatocellular carcinoma (HCC) has been registered. Our aim is to reduce inequalities in diagnosis and treatment and to offer patients univocal recommendations in any institution.The members of the panel - gastroenterologists, radiologists, surgeons and oncologists -were selected on the basis of their publication records and expertise. Thirteen clinical questions, derived from clinical needs, and an integration of all the committee members' suggestions, were formulated. Modified Delphi approach involving a detailed literature review and the collective judgement of experts, was applied to this work.Thirteen statements were derived from expert opinions' based on the current literature, on recently developed reviews and on technological advancements. Each statement is discussed in a short paragraph reporting the current key evidence. As this is an emerging issue, the number of papers on HCC in beta-thalassemia patients is limited and based on anecdotal cases rather than on randomized controlled studies. Therefore, the panel has discussed, step by step, the possible differences between beta-thalassemia and non beta-thalassemia patients. Despite the paucity of the literature, practical and concise statements were generated.This paper offers a practical guide organized by statements describing how to manage HCC in patients with beta-thalassemia.
β-地中海贫血症是一组遗传性血红蛋白疾病，是世界上最常见的遗传疾病之一，在地中海盆地尤为常见。随着β-地中海贫血症患者的生存时间延长，以前未知的并发症的发生频率也越来越高。其中，肝细胞癌（HCC）的风险增加已得到证实。我们的目标是减少诊断和治疗方面的不平等，并在任何机构为患者提供明确的建议。
小组成员 - 胃肠病学家、放射科医生、外科医生和肿瘤学家 - 根据他们的出版物记录和专业知识进行了选择。从临床需求中得出了 13 个临床问题，并整合了所有委员会成员的建议。采用改良 Delphi 方法，对文献进行了详细的回顾，并对专家的集体判断进行了应用。
从专家意见中得出了 13 项基于当前文献、最近的综述和技术进步的声明。每个声明都在一个简短的段落中进行了讨论，报告了当前的关键证据。由于这是一个新兴问题，关于β-地中海贫血症患者 HCC 的论文数量有限，并且基于轶事案例而不是随机对照研究。因此，小组逐步讨论了β-地中海贫血症和非β-地中海贫血症患者之间可能存在的差异。尽管文献有限，但仍提出了实用和简明的声明。
本文提供了一个实用指南，通过描述如何管理β-地中海贫血症患者的 HCC 的陈述进行组织。
