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Medline ® Abstract for Reference 91

of 'Red blood cell transfusion in sickle cell disease'

Tissue iron evaluation in chronically transfused children shows significant levels of iron loading at a very young age.
Berdoukas V, Nord A, Carson S, Puliyel M, Hofstra T, Wood J, Coates TD
Am J Hematol. 2013 Nov;88(11):E283-5. Epub 2013 Sep 2.
Chronic blood transfusions start at a very young age in subjects with transfusion-dependent anemias, the majority of whom have hereditary anemias. To understand how rapidly iron overload develops, we retrospectively reviewed 308 MRIs for evaluation of liver, pancreatic, or cardiac iron in 125 subjects less than 10 years old. Median age at first MRI evaluation was 6.0 years. Median liver iron concentrations in patients less than 3.5 years old were 14 and 13 mg/g dry weight in thalassemia major (TM) and Diamond-Blackfan anemia (DBA) patients, respectively. At time of first MRI, pancreatic iron was markedly elevated (>100 Hz) in DBA patients, and cardiac iron ( R2*>50 Hz) was present in 5/112 subjects (4.5%), including a 2.5 years old subject with DBA. Five of 14 patients (38%) with congenital dyserythropoietic anemia (CDA) developed excess cardiac iron before their 10th birthday. Thus, clinically significant hepatic and cardiac iron accumulation occurs at an early age in patients on chronic transfusions, particularly in those with ineffective or absent erythropoiesis, such as DBA, CDA, and TM, who are at higher risk for iron cardiomyopathy. Performing MRI for iron evaluation in the liver, heart, and pancreas as early as feasible, particularly in those conditions in which there is suppressed bone marrow activity is very important in the management of iron loaded children in order to prescribe appropriate chelation to prevent long-term sequelae. Am. J. Heamtol. 88:E283-E285, 2013.©2013 Wiley Periodicals, Inc.
Division of Hematology/Oncology, Department of Pediatrics, Keck School of Medicine, Children's Hospital Los Angeles, University of Southern California, Los Angeles, California.