T2* MRI in regularly transfused children with thalassemia intermedia: Serum ferritin does not reflect liver iron stores

Pediatric Hematology and Oncology

Volumn 29, Issue 6, 2012, Pages 579-584

  Tony, Surekha ^a   Daar, Shahina ^a   Elshinawy, Mohamed ^a   Al Zadjaly, Shoaib ^a   Al Khabori, Murtadha ^a   Wali, Yasser ^a  

^a SULTAN QABOOS UNIVERSITY HOSPITAL   (Oman)

Author keywords

Children; Iron overload; MRI; Serum ferritin; T2; Thalassemia intermedia

Indexed keywords

DEFERIPRONE; DEFEROXAMINE; FERRITIN; IRON;

ADOLESCENT; ARTICLE; CHILD; CLINICAL ARTICLE; DRUG DOSE ESCALATION; FEMALE; FERRITIN BLOOD LEVEL; HUMAN; IRON OVERLOAD; MALE; NUCLEAR MAGNETIC RESONANCE IMAGING; PRESCHOOL CHILD; SCHOOL CHILD; THALASSEMIA INTERMEDIA; TRANSFUSION; BETA THALASSEMIA; BLOOD; BLOOD TRANSFUSION; LIVER; METABOLISM;

ADOLESCENT; BETA-THALASSEMIA; BLOOD TRANSFUSION; CHILD; CHILD, PRESCHOOL; FEMALE; FERRITINS; HUMANS; IRON; IRON OVERLOAD; LIVER; MAGNETIC RESONANCE IMAGING; MALE;

EID: 84865346929     PISSN: 08880018     EISSN: 15210669     Source Type: Journal    
DOI: 10.3109/08880018.2012.708891     Document Type: Article

References (28)

1. Olivieri NF. The β-thalassemias. N Engl J Med. 1999;341:99-109.
2. Taher A, Musallam KM, Karimi M, et al. Overview of practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the optimal care study. Blood. 2010;115(10):1886-1892.
3. Gardenghi S, Ramos P, Follenzi A, et al. Hepcidin and Hfe in iron overload in beta thalassemia. Ann N Y Acad Sci. 2010;1202:221-225.
4. Anderson LJ, Holden S, Davis B, et al. Cardiovascular T2-star (T2*)magnetic resonance for the early diagnosis ofmyocardial iron overload. Eur Heart J. 2001;22:2171-2179. (Pubitemid 33139463)
5. Taher A, Ismaeel H, Cappellini MD. Thalassaemia intermedia: revisited. Blood Cells Mol Dis. 2006;37(1):12-20.
6. Nemeth E. Hepcidin in beta-thalassemia. Ann N Y Acad Sci. 2010;1202:31-35.
7. Kearney SL, Nemeth E, Neufeld EJ, et al. Urinary hepcidin in congenital chronic anemias. Pediatr Blood Cancer. 2007;48(1):57-63. (Pubitemid 44871594)
8. Origa R, Galanello R, Ganz T, et al. Liver iron concentrations and urinary hepcidin in betathalassemia. Haematologica. 2007;92(5):583-588. (Pubitemid 350144260)
9. Cappellini MD, Cohen A, Eleftheriou A, et al. Guidelines for the Clinical Management of Thalassaemia. 2nd rev.ed. Nicosia, Cyprus:Thalassaemia International Federation; 2008.
10. Aessopos A, Farmakis D, Deftereos S, et al.Thalassemia heart disease: a comparative evaluation of thalassemiamajor and thalassemia intermedia. Chest. 2005;127(5):1523-1530. (Pubitemid 46218775)
11. Isma'eel H, Chafic AH, Rassi FE, et al. Relation between iron-overload indices, cardiac echo-Doppler and biochemicalmarkers in thalassemia intermedia. Am J Cardiol. 2008;102(3):363-367.
12. Borgna-Pignatti C,Marsella M, Zanforlin N.The natural history of thalassemia intermedia. Ann N Y Acad Sci. 2010;1202:214-220.
13. Westwood M, Anderson LJ, Firmin DN, et al. A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging. 2003;18(1):33-39. (Pubitemid 36791266)
14. Phylipsen M, Prior JF, Lim E, et al. Two new alpha1-globin gene point mutations: Hb Nedlands (HBA1:c.86C>T) [alpha28(B9)Ala->Val] and Hb Queens Park (HBA1:c.98T>A) [alpha32(B13)Met->Lys] Hemoglobin. 2010 34 2 123-126.
15. Laosombat V, Viprakasit V, Chotsampancharoen T, et al. Clinical features and molecular analysis in Thai patients with HbH disease. Ann Hematol. 2009;88(12):1185-1192.
16. dos Santos CO, Zhou S, Secolin R, et al. Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function. Am J Hematol. 2008;83(2):103-108.
17. So CC, So AC, Chan AY, et al. Detection and characterisation of beta-globin gene cluster deletions in Chinese using multiplex ligation-dependent probe amplification. J Clin Pathol. 2009;62(12):1107-1111.
18. Ganz T. Hepcidin, a key regulator of iron metabolism and mediator of anemia of inflammation. Blood. 2003;102(3):783-788. (Pubitemid 36917762)
19. Gardenghi S, Grady RW, Rivella S. Anemia, ineffective erythropoiesis, and hepcidin: interacting factors in abnormal iron metabolism leading to iron overload in β-thalassemia. Hematol Oncol Clin North Am. 2010;24(6):1089-1107.
20. Pakbaz Z, Fischer R, Fung E, et al. Serumferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients. Pediatr Blood Cancer. 2007;49(3):329-332. (Pubitemid 47236225)
21. Olthof AW, Sijens PE, Kreeftenberg HG, et al. Correlation between serumferritin levels and liver iron concentration determined by MR imaging: impact of hematologic disease and inflammation. Magn Reson Imaging 2007;25(2):228-231. (Pubitemid 46162057)
22. Nielsen P, Günther U, DürkenM, et al. Serumferritin iron in iron overload and liver damage: correlation to body iron stores and diagnostic relevance. J Lab Clin Med. 2000;135(5):413-418.
23. Tanno T, Bhanu NV, Oneal PA, et al. High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin. NatMed. 2007;13(9):1096-1101. (Pubitemid 47517510)
24. Finkenstedt A, Bianchi P, Theurl I, et al. Regulation of iron metabolism through GDF15 and hepcidin in pyruvate kinase deficiency. Br J Hematol. 2009;144(5):789-793.
25. Wali Y, Al Zadjali S, Elshinawy M, et al. Severity ranking of non-deletional alpha thalassemic alleles: insights from an Omani family study. Eur J Haematol. 2011;86(6):507-511.
26. Roghi A, Cappellini MD,Wood JC, et al. Absence of cardiac siderosis despite hepatic iron overload in Italian patients with thalassemia intermedia: anMRI T2* study. Ann Hematol. 2010;89(6):585-589.
27. Origa R, Barella S, Argiolas GM, et al. No evidence of cardiac iron in 20 never- or minimallytransfused patients with thalassemia intermedia. Haematologica. 2008;93(7):1095-1096. (Pubitemid 351957030)
28. Jensen PD, Jensen FT, Christensen T, et al. Evaluation of myocardial iron by magnetic resonance imaging during iron chelation therapywith deferrioxamine: indication of close relation betweenmyocardial iron content and chelatable iron pool. Blood. 2003;101:4632-4639. (Pubitemid 36857840)