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Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease.
Br J Haematol. 2016 Nov; 175(4):705-713.BJ

Abstract

Transfusional iron overload represents a substantial challenge in the management of patients with sickle cell disease (SCD) who receive chronic or episodic red blood cell transfusions. Iron-induced cardiomyopathy is a leading cause of death in other chronically transfused populations but rarely seen in SCD. Study objectives were to: (i) examine the extent of myocardial and hepatic siderosis using magnetic resonance imaging (MRI) in chronically transfused SCD patients, and (ii) evaluate the relationship between long-term (over the 5 years prior to enrolment) mean serum ferritin (MSF), spot-ferritin values and liver iron content (LIC) measured using MRI and liver biopsy. Thirty-two SCD patients (median age 15 years) with transfusional iron overload were recruited from two U.S. institutions. Long-term MSF and spot-ferritin values significantly correlated with LIC by MRI-R2* (r = 0·77, P < 0·001; r = 0·82, P < 0·001, respectively). LIC by MRI-R2* had strong positive correlation with LIC by liver biopsy (r = 0·98, P < 0·001) but modest inverse correlation with cardiac MRI-T2* (r = -0·41, P = 0·02). Moderate to severe transfusional iron overload in SCD was not associated with aberrations in other measures of cardiac function based on echocardiogram or serum biomarkers. Our results suggest that SCD patients receiving chronic transfusions may not demonstrate significant cardiac iron loading irrespective of ferritin trends, LIC and erythropoiesis suppression.

Authors+Show Affiliations

Department of Pediatrics, Feinberg School of Medicine at Northwestern University, Chicago, IL, USA. Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.Department of Pediatrics, Feinberg School of Medicine at Northwestern University, Chicago, IL, USA. Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.Division of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA. Department of Radiology, Feinberg School of Medicine at Northwestern University, Chicago, IL, USA.Department of Pediatrics, Division of Hematology and Oncology, University of Illinois School of Medicine at Chicago, Chicago, IL, USA.Department of Pediatrics, Feinberg School of Medicine at Northwestern University, Chicago, IL, USA. Division of Pediatric Cardiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.Department of Pediatrics, Feinberg School of Medicine at Northwestern University, Chicago, IL, USA. Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

27507431

Citation

Badawy, Sherif M., et al. "Assessing Cardiac and Liver Iron Overload in Chronically Transfused Patients With Sickle Cell Disease." British Journal of Haematology, vol. 175, no. 4, 2016, pp. 705-713.
Badawy SM, Liem RI, Rigsby CK, et al. Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease. Br J Haematol. 2016;175(4):705-713.
Badawy, S. M., Liem, R. I., Rigsby, C. K., Labotka, R. J., DeFreitas, R. A., & Thompson, A. A. (2016). Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease. British Journal of Haematology, 175(4), 705-713. https://doi.org/10.1111/bjh.14277
Badawy SM, et al. Assessing Cardiac and Liver Iron Overload in Chronically Transfused Patients With Sickle Cell Disease. Br J Haematol. 2016;175(4):705-713. PubMed PMID: 27507431.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease. AU - Badawy,Sherif M, AU - Liem,Robert I, AU - Rigsby,Cynthia K, AU - Labotka,Richard J, AU - DeFreitas,R Andrew, AU - Thompson,Alexis A, Y1 - 2016/08/10/ PY - 2016/03/25/received PY - 2016/06/09/accepted PY - 2016/8/11/pubmed PY - 2017/5/16/medline PY - 2016/8/11/entrez KW - chronic transfusions KW - iron overload KW - liver biopsy KW - magnetic resonance imaging KW - sickle cell disease SP - 705 EP - 713 JF - British journal of haematology JO - Br J Haematol VL - 175 IS - 4 N2 - Transfusional iron overload represents a substantial challenge in the management of patients with sickle cell disease (SCD) who receive chronic or episodic red blood cell transfusions. Iron-induced cardiomyopathy is a leading cause of death in other chronically transfused populations but rarely seen in SCD. Study objectives were to: (i) examine the extent of myocardial and hepatic siderosis using magnetic resonance imaging (MRI) in chronically transfused SCD patients, and (ii) evaluate the relationship between long-term (over the 5 years prior to enrolment) mean serum ferritin (MSF), spot-ferritin values and liver iron content (LIC) measured using MRI and liver biopsy. Thirty-two SCD patients (median age 15 years) with transfusional iron overload were recruited from two U.S. institutions. Long-term MSF and spot-ferritin values significantly correlated with LIC by MRI-R2* (r = 0·77, P < 0·001; r = 0·82, P < 0·001, respectively). LIC by MRI-R2* had strong positive correlation with LIC by liver biopsy (r = 0·98, P < 0·001) but modest inverse correlation with cardiac MRI-T2* (r = -0·41, P = 0·02). Moderate to severe transfusional iron overload in SCD was not associated with aberrations in other measures of cardiac function based on echocardiogram or serum biomarkers. Our results suggest that SCD patients receiving chronic transfusions may not demonstrate significant cardiac iron loading irrespective of ferritin trends, LIC and erythropoiesis suppression. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/27507431/Assessing_cardiac_and_liver_iron_overload_in_chronically_transfused_patients_with_sickle_cell_disease_ L2 - https://doi.org/10.1111/bjh.14277 DB - PRIME DP - Unbound Medicine ER -