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2013, Number 1

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Ann Hepatol 2013; 12 (1)

Hepatocellular carcinoma in hepatitis-negative patients with thalassemia intermedia: a closer look at the role of siderosis

Maakaron JE, Cappellini MD, Graziadei G, Ayache JB, Taher AT

Language: English
References: 22
Page: 142-146
PDF size: 258.36 Kb.


ABSTRACT

Patients with thalassemia are often exposed to several risk factors for developing hepatocellular carcinoma (HCC) due to their repeated transfusions. However, even transfusion-independent patients with thalassemia intermedia (TI) can develop HCC, which is mainly attributed to a state of iron overload. We report here two cases and review the literature for the association between TI and HCC. Along with our cases, a total of 36 cases of HCC in thalassemic patients were reported in the literature. Of these, 22 (61%) were TI patients with 6 (27%) of them being hepatitis B and C negative. There was no consistency in their characteristics; therefore, we recommended screening thresholds for HCC in TI patients based on their total liver iron concentration (LIC).


REFERENCES

  1. Taher AT, Musallam KM, Cappellini MD, Weatherall DJ. Optimal management of beta thalassaemia intermedia. Br J Haematol 2011; 512-23.

  2. Taher A, Musallam KM, El Rassi F, Duca L, Inati A, Koussa S, Cappellini MD. Levels of non-transferrin-bound iron as an index of iron overload in patients with thalassaemia intermedia. Br J Haematol 2009; 569-72.

  3. Taher A, El Rassi F, Isma’eel H, Koussa S, Inati A, Cappellini MD. Correlation of liver iron concentration determined by R2 magnetic resonance imaging with serum ferritin in patients with thalassemia intermedia. Haematologica 2008: 1584-6.

  4. Origa R, Galanello R, Ganz T, Giagu N, Maccioni L, Faa G, Nemeth E. Liver iron concentrations and urinary hepcidin in beta-thalassemia. Haematologica 2007; 583-8.

  5. Taher A, Hershko C, Cappellini MD. Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies. Br J Haematol 2009; 634-40.

  6. Kowdley KV. Iron, hemochromatosis, and hepatocellular carcinoma. Gastroenterology 2004; S79-S86.

  7. Mancuso A, Rigano P, Renda D, Di Salvo V, Pignatti CB, Guddo F, Buccellato A, et al. Hepatocellular carcinoma on cirrhosis-free liver in a HCV-infected thalassemic. Am J Hematol 2005; 158-9.

  8. Mancuso A. Hepatocellular carcinoma in thalassemia: A critical review. World J Hepatol 2010; 171-4.

  9. Bradbear RA, Bain C, Siskind V, Schofield FD, Webb S, Axelsen EM, Halliday JW, et al. Cohort study of internal malignancy in genetic hemochromatosis and other chronic nonalcoholic liver diseases. J Natl Cancer Inst 1985; 81-4.

  10. Fleming RE, Sly WS. Mechanisms of iron accumulation in hereditary hemochromatosis. Annu Rev Physiol 2002; 663-80.

  11. Pietrangelo A. Hemochromatosis: an endocrine liver disease. Hepatology 2007; 1291-301.

  12. Chung H, Kudo M, Kawasaki T, Kitano M, Minami Y, Suetomi Y, Onda H. Hepatocellular carcinoma associated with secondary haemochromatosis in non-cirrhotic liver: a case report. Hepatol Res 2003; 254-8.

  13. Stevens RG, Jones DY, Micozzi MS, Taylor PR. Body iron stores and the risk of cancer. N Engl J Med 1988; 1047-52.

  14. Herrinton LJ, Friedman GD, Baer D, Selby JV. Transferrin saturation and risk of cancer. Am J Epidemiol 1995; 692-8.

  15. Moyo VM, Makunike R, Gangaidzo IT, Gordeuk VR, McLaren CE, Khumalo H, Saungweme T, et al. African iron overload and hepatocellular carcinoma (HA-7-0-080). Eur J Haematol 1998; 28-34.

  16. Turlin B, Juguet F, Moirand R, Le Quilleuc D, Loreal O, Campion JP, Launois B, et al. Increased liver iron stores in patients with hepatocellular carcinoma developed on a noncirrhotic liver. Hepatology 1995; 446-50.

  17. Ioannou GN, Kowdley KV. Iron, HFE mutations, and hepatocellular carcinoma: is hepatic iron a carcinogen? Clin Gastroenterol Hepatol 2003; 246-8.

  18. Borgna-Pignatti C, Vergine G, Lombardo T, Cappellini MD, Cianciulli P, Maggio A, Renda D, et al. Hepatocellular carcinoma in the thalassaemia syndromes. Br J Haematol 2004; 114-7.

  19. Mancuso A, Sciarrino E, Renda MC, Maggio A. A prospective study of hepatocellular carcinoma incidence in thalassemia. Hemoglobin 2006; 119-24.

  20. Restivo Pantalone G, Renda D, Valenza F, D’Amato F, Vitrano A, Cassara F, Rigano P, et al. Hepatocellular carcinoma in patients with thalassaemia syndromes: clinical characteristics and outcome in a long term single centre experience. Br J Haematol 2010; 245-7.

  21. Bruix J, Sherman M. Management of hepatocellular carcinoma: an update. Hepatology 2011; 1020-2.

  22. Musallam KM, Cappellini MD, Wood JC, Motta I, Graziadei G, Tamim H, Taher AT. Elevated liver iron concentration is a marker of increased morbidity in patients with beta thalassemia intermedia. Haematologica 2011; 1605-12.




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