Borgna-Pignatti Caterina, Garani Maria Chiara, Forni Gian Luca, Cappellini Maria Domenica, Cassinerio Elena, Fidone Carmelo, Spadola Vincenzo, Maggio Aurelio, Restivo Pantalone Gaetano, Piga Antonio, Longo Filomena, Gamberini Maria Rita, Ricchi Paolo, Costantini Silvia, D'Ascola Domenico, Cianciulli Paolo, Lai Maria Eliana, Carta Maria Paola, Ciancio Angela, Cavalli Paola, Putti Maria Caterina, Barella Susanna, Amendola Giovanni, Campisi Saveria, Capra Marcello, Caruso Vincenzo, Colletta Grazia, Volpato Stefano
Dipartimento di Scienze Mediche-Pediatria, Università di Ferrara, Ferrara, Italy.
Br J Haematol. 2014 Oct;167(1):121-6. doi: 10.1111/bjh.13009. Epub 2014 Jul 3.
The risk of developing hepatocellular carcinoma (HCC) in patients with thalassaemia is increased by transfusion-transmitted infections and haemosiderosis. All Italian Thalassaemia Centres use an ad hoc form to report all diagnoses of HCC to the Italian Registry. Since our last report, in 2002, up to December 2012, 62 new cases were identified, 52% of whom were affected by thalassaemia major (TM) and 45% by thalassaemia intermedia (TI). Two had sickle-thalassaemia (ST). The incidence of the tumour is increasing, possibly because of the longer survival of patients and consequent longer exposure to the noxious effects of the hepatotropic viruses and iron. Three patients were hepatitis B surface antigen-positive, 36 patients showed evidence of past infection with hepatitis B virus (HBV). Fifty-four patients had antibodies against hepatitis C virus (HCV), 43 of whom were HCV RNA positive. Only 4 had no evidence of exposure either to HCV or HBV. The mean liver iron concentration was 8 mg/g dry weight. Therapy included chemoembolization, thermoablation with radiofrequency and surgical excision. Three patients underwent liver transplant, 21 received palliative therapy. As of December 2012, 41 patients had died. The average survival time from HCC detection to death was 11·5 months (1·4-107·2 months). Ultrasonography is recommended every 6 months to enable early diagnosis of HCC, which is crucial to decrease mortality.
地中海贫血患者发生肝细胞癌(HCC)的风险因输血传播感染和血色素沉着症而增加。所有意大利地中海贫血中心都使用一种专门表格向意大利登记处报告所有HCC诊断病例。自我们2002年的上次报告以来,截至2012年12月,共发现62例新病例,其中52%为重型地中海贫血(TM)患者,45%为中间型地中海贫血(TI)患者。2例为镰状细胞-地中海贫血(ST)患者。肿瘤的发病率在上升,这可能是因为患者生存期延长,从而更长时间暴露于嗜肝病毒和铁的有害影响之下。3例患者乙肝表面抗原呈阳性,36例有既往感染乙肝病毒(HBV)的证据。54例患者有抗丙型肝炎病毒(HCV)抗体,其中43例HCV RNA呈阳性。只有4例没有接触HCV或HBV的证据。肝脏铁浓度平均为8毫克/克干重。治疗方法包括化疗栓塞、射频热消融和手术切除。3例患者接受了肝移植,21例接受了姑息治疗。截至2012年12月,41例患者死亡。从检测出HCC到死亡的平均生存时间为11.5个月(1.4 - 107.2个月)。建议每6个月进行一次超声检查,以便早期诊断HCC,这对于降低死亡率至关重要。