Hepatic iron overload and fibrosis in patients with beta thalassemia major after hematopoietic stem cell transplantation: A pilot study.

  • Ardeshir Ghavamzadeh Mail Professor of Medicine, Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Mehrzad Mirzania Hematologist and Medical Oncologist, Cancer Research Center, Cancer Institute, Tehran University of medical Sciences, Tehran, Iran.
  • Naser Kamalian Professor of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
  • Nahid Sedighi Associate Professor of Medicine, Advanced Diagnostic and Interventional Radiology, MSK research group, Tehran University of Medical Sciences, Tehran, Iran.
  • Parisima Azimi Tehran University of Medical Sciences, Tehran, Iran.
Keywords:
Hematopoietic Stem Cell Transplantation, Hepatic Iron Overload, Liver Fibrosis, Perls’ Prussian Blue Staining

Abstract

Currently, hematopoietic stem cell transplantation (HSCT) is the only curative option for patients with beta-thalassemia major, but liver iron overload in these patients will not decrease and hepatic fibrosis may still progress despite successful HSCT. Liver biopsy samples were taken from 14 patients (Out of 25 patients) who underwent HSCT. All patients met three criteria: negative HCV antibody, liver fibrosis in samples before HSCT and lack of regular treatment for iron overload after HSCT (Because patients did not consent to phlebotomy or they had not regular follow-up). We evaluated liver fibrosis and liver iron overload by a semi quantitative method, Perls' Prussian blue staining, before and after HSCT. HSCT was successful in all the patients. Liver iron overload did not change after transplant (P=0.61), but hepatic fibrosis progressed after transplant (P=0.01). In patients with beta thalassemia major who previously had some degree of liver fibrosis, HSCT alone cannot reduce liver iron overload and liver fibrosis will increase. We recommend that regardless of the amount of iron overload in patients with beta thalassemia major that have shown some degree of fibrosis in their liver biopsy before transplantation, appropriate steps should be taken to reduce iron overload as soon as possible after successful transplantation.

References

Caterina B, Pignatti G. Thalassemia and Related Disorders. In: John PG, John F, editors. Wintrobe's Clinical Hematology. 11ed: LWW 2004;P1319-1365.

O'Brien RT. Iron overload: clinical and pathologic aspect in pediatrics. Semin Hematol. 1977; 14:115-125.

Bacon B, Britton R. The pathology of hepatic iron overload: a free radical-mediated process? Hepatology. 1990; 11: 127–137,

Bacon B, O’Neill R, Britton R. Hepatic mitochondrial energy production in rats with chronic iron overload. Gastroenterology. 1993;105:1134–1140.

Muretto P, Angelucci E, Lucarelli G. Reversibility of cirrhosis in patients cured of thalassemia by bone marrow transplantation. Ann Intern Med. 2002;136:667-672.

George DK, Goldwurm S, Macdonalds GA, et al. Increased hepatic iron concentration in nonalcoholic steatohepatitis is associated with increased fibrosis. Gastroenterology. 1998,114:311–318,.

Maurer HS, Lloyd-Still JD, Ingrisano C, et al. A prospective evaluation of iron chelation therapy in children with severe beta-thalassemia. A six-year study. Am J Dis Child. 1988;142:287-292.

Angelucci E, Ripalti M, Barociani D, et al. Phlebotomy to reduce iron over-load in patients cured of thalassemia by marrow transplantation. Bone Marrow Transplant. 1997; 19 Suppl 2 .

Younossi ZM, Gramlich T, Bacon BR, et al. Hepatic iron and nonalcoholic fatty liver disease.Hepatology. 1999 Oct; 30(4):847-50.

Barosi G, Arbustini E, Gavazzi A, et al. Myocardial iron grading by endomyocardial biopsy. A clinico-pathologic study on iron overloaded patients. Eur J Haematol. 1989; Apr;42(4):382-8.

Lucarelli G, Andreani M, Angelucci E. The cure of the thalassemia with bone marrow transplantation. Bone Marrow Transplant. 2001;28:S11-13.

Angelucci E, Baronciani D, Lucarelli G, et al. Needle liver biopsy in thalassaemia: Analyses of diagnostic accuracy and safety in 1184 consecutive biopsies. Br J Haematol. 1995;89:757,.

Soriano-Cubells MJ, Aparisi-Querada L. Rapid determination of copper, iron and zinc in liver biopsies. Atomic Spectroscopy. 1984, 5:217,.

Olynyk JK, Bacon BR. Hereditary hemochromatosis. Detecting and correcting iron overload. Postgrad Med. 1994;96:151,.

Giardini C, Galimberti M, Lucarelli G, et al. Desferrioxamine therapy accelerates clearance of iron deposits after bone marrow transplantation for thalassaemia. Br J Haematol. 1955; 89:868-873.

Lucarelli G, Galimberti M, Polchi P, et al. Marrow transplantation in patients with Thalassemia responsive to iron chelation therapy. N Engl J Med. 1993;329:840,.

Lucarelli G, Angelucci E, Giardini C, et al. Fate of iron stores in thalassemia after bone marrow transplantation. Lancet. 1993;342:1388,.

Muretto P, Del Fiasco S, Angelucci E, et al. Bone marrow transplantation in thalassemia: Modification of hepatic iron overload and related pathologies after long-term engrafting. Liver. 1994; 14:14.

How to Cite
1.
Ghavamzadeh A, Mirzania M, Kamalian N, Sedighi N, Azimi P. Hepatic iron overload and fibrosis in patients with beta thalassemia major after hematopoietic stem cell transplantation: A pilot study. Int J Hematol Oncol Stem Cell Res. 9(2):55-59.
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