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Abstract. Introduction: Sickle cell disease (SCD) is an important cause of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time which challenge its management. Aim of our study: To determine the iron status of 17 patients with NT-SCD patients and 6 patients with TD- SCD using both serum ferritin level (SF ) and Ferriscan® evaluation of liver iron content (LIC) and correlate values of LIC on the one hand with SF levels and some hepatic functions (ALT, AST, ALP and albumin). Results: 17 adults with NT-SCD (n = 17, age: 32±15 years) were studied. Seven of NT-SCD had serum ferritin > 500 μg/L, 4 out of the seven had high liver iron measured by FerriScan® (> 30 mg/kg/ tissue dry weight - DW). Two patients had high liver iron content despite a concomitant serum ferritin concentration < 500 μg/L. Two patients had high serum ferritin (1.117 μg/L and 675 μg/L) while their LIC was normal (< 30 mg/kg/DW). 5 patients had elevated ALT and/or AST concentrations. In TD-SCD (n = 6, age = 25 ±11 years), 2 patients had serum ferritin <500 μg/L, one of them had high LIC (127 mg/kg/DW). Liver enzymes were high in two patients. Serum ferritin concentration was correlated significantly with LIC (r = 0.85, p < 0.001). Neither serum ferritin level, nor LIC was correlated significantly with hepatic enzyme levels. Conclusions: A significant number of our patients with ND-SCD had high LIC , high serum ferritin and elevated hepatic enzymes (ALT and AST). Despite some limitations of our study (small NT-SCD cohort), these findings have important clinical implications. We recommend to measure serum ferritin and LIC in NT-SCD patients to apply therapeutic preventive measures with iron chelation therapy in patients with high LIC.
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1.Porter J ,Garbowski M. Consequences and management of iron overload in sickle cell disease. ASH Education Book December 6, 2013 vol. 2013 no. 1 447-456. http:// ash education book.hematologylibrary.org/content/2013/1/447.full
2.Mohanty D, Mukherjee MB, Colah RB, Wadia M, Ghosh K, Chottray GP, Jain D, Italia Y, Ashokan K, Kaul R, Shukla DK, Muthuswamy V. Iron deficiency anaemia in sickle cell disorders in India. Indian J Med Res 2008; 127:366–369.
3.Walter PB,Harmatz P, Vichinsky E. Iron metabolism and iron chelation in sickle cell disease. Acta Haematol 2009;122:174–183.
4. Nielsen P, Engelhardt R, Düllmann J, Fischer R. Non-Invasive Liver Iron Quantification by SQUID-Biosusceptometry and Serum Ferritin Iron as New Diagnostic Parameters in Hereditary Hemochromatosis. Blood Cells Mol Dis 2002;29:451-458.
5.Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, Casella JF, Alvarez OA, Barredo JC, Lee MT, Iyer RV, Kutlar A, McKie KM, McKie V, Odo N, Gee B, Kwiatkowski JL, Woods GM, Coates T, Wang W, Adams RJ. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 2009;114:4632-4638.
6.Brown K, Subramony C, May W, Megason G, Liu H, Bishop P, Walker T, Nowicki MJ. Hepatic iron overload in children with sickle cell anemia on chronic transfusion therapy. J Pediatr Hematol Oncol 2009;31:309-312.
7.Harmatz P, Butensky E, Quirolo K, Williams R, Ferrell L, Moyer T, Golden D, Neumayr L, Vichinsky E. Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy. Blood 2000;96:76-79.
8.Bassett ML, Halliday JW, Powell LW. Value of hepatic iron measurements in early hemochromatosis and determination of the critical iron level associated with fibrosis. Hepatology 1986;6:24-29.
9. Angelucci E, Baronciani D, Lucarelli G, Baldassarri M, Galimberti M, Giardini C, Martinelli F, Polchi P, Polizzi V, Ripalti M, Needle liver biopsy in thalassaemia: analyses of diagnostic accuracy and safety in 1184 consecutive biopsies. Br J Haematol 1995;89:757-761.
10. St Pierre TG, Clark PR, Chua-Anusorn W, Fleming AJ, Jeffrey GP, Olynyk JK, Pootrakul P, Robins E, Lindeman R. Noninvasive measurement and imaging of liver iron concentrations using proton magnetic resonance. Blood 2005;105:855–861.
11.Hankins JS, McCarville MB, Loeffler RB, Smeltzer MP, Onciu M, Hoffer FA, Li CS, Wang WC, Ware RE, Hillenbrand CM. R2* magnetic resonance imaging of the liver in patients with iron overload. Blood 2009;113:4853–4855.
12.Inati A. Recent advances in improving the management of sickle cell disease. Blood Rev 2009;23 (Suppl 1):S9-13.
13. Casale M, Meloni A, Filosa A, Cuccia L, Caruso V, Palazzi G, Gamberini MR, Pitrolo L, Putti MC, D'Ascola DG, Casini T, Quarta A, Maggio A, Neri MG, Positano V, Salvatori C, Toia P, Valeri G, Midiri M, Pepe A. Multiparametric Cardiac Magnetic Resonance Survey in Children With Thalassemia Major: A Multicenter Study.Circ Cardiovasc Imaging. 2015 Aug;8(8):e003230.
14. Positano V, Pepe A, Santarelli MF, Scattini B, De Marchi D, Ramazzotti A, Forni G, Borgna-Pignatti C, Lai ME, Midiri M, Maggio A, Lombardi M, Landini L. Standardized T2* map of normal human heart in vivo to correct T2* segmental artefacts. NMR Biomed 2007;20:578–590.
15.Vichinsky E, Butensky E, Fung E, Hudes M, Theil E, Ferrell L, Williams R, Louie L, Lee PD, Harmatz P. Comparison of organ dysfunction in transfused patients with SCD or beta thalassemia.Am J Hematol. 2005;80:70-74.
16.Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, Wang WC, Hoppe C, Hagar W, Darbari DS, Malik P. Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management. Scientific World Journal. 2012;2012:949535
17.Drasar E, Vasavda N, Igbineweka N, Awogbade M, Allman M, Thein SL. Serum ferritin and total units transfused for assessing iron overload in adults with sickle cell disease. Br J Haematol 2012;157:645-647.
18.Banerjee S, Owen C, Chopra S. Sickle cell hepatopathy. Hepatology. 2001;33:1021-1028.
19.Lucania G, Vitrano A, Filosa A,Maggio A.Chelation treatment in sickle-cell-anaemia: much ado about nothing? Br J Haematol.2011; 154:545–555.
20. Siegelman ES, Mitchell DG, Semelka RC. Abdominal iron deposition: metabolism,
MR fi ndings, and clinical importance. Radiology. 1996;199:13-22.
21. Spina JC, Alvarez del Rivero MA, Kidd C, Pietrani M, Savluky L, García Mónaco RD. Noninvasive assessment of hepatic iron overload in patients with hemochromatosis Rev Argent Radiol 2013;77:139-146