title: Cardiac iron overload in sickle-cell disease.
abstract: Chronically transfused sickle cell disease (SCD) patients have lower risk of myocardial iron overload (MIO) than comparably transfused thalassemia major (TM) patients. However, cardioprotection is incomplete. We present the clinical characteristics of six patients who have prospectively developed MIO, to identify potential risk factors for cardiac iron accumulation. From 2002 to 2011, cardiac, hepatic, and pancreatic iron overload were assessed by R2 and R2 * magnetic resonance imaging techniques in 201 chronic transfused SCD patients as part of their clinical care. At the time, they developed MIO, five of six patients had been on chronic transfusion for more than 11 years; only one was on exchange transfusion. The time to MIO was correlated with reticulocyte and hemoglobin S percentages. All patients had qualitatively poor chelation compliance (<50%). All patients had serum ferritin levels >4600 ng/ml and liver iron concentration >22 mg/g. Pancreatic R2 * was >100 Hz in every patient studied (5/6). Cardiac iron rose proportionally to pancreas R2 *, with all patients having pancreas R2 *>100 Hz when cardiac iron was present. MIO had a threshold relationship with liver iron that was higher than observed in TM patients. In conclusion, MIO occurs in a small percentage of chronically transfused SCD patients and is only associated with exceptionally poor control of total body iron stores. Duration of chronic transfusion is clearly important but other factors, such as levels of effective erythropoiesis, appear to contribute to cardiac risk. Pancreas R2 * can serve as a valuable screening tool for cardiac iron in SCD patients.
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Antonella
authors.initials: A
authors.lastname: Meloni
authors.forename: Mammen
authors.initials: M
authors.lastname: Puliyel
authors.forename: Alessia
authors.initials: A
authors.lastname: Pepe
authors.forename: Vasili
authors.initials: V
authors.lastname: Berdoukas
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Coates
authors.forename: John C
authors.initials: JC
authors.lastname: Wood
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000378:metabolism* / Q000473:pathology / Q000628:therapy; D001803:Blood Transfusion / Q000379:methods; D005260:Female; D006801:Humans; D007501:Iron / Q000097:blood / Q000378:metabolism*; D019190:Iron Overload / Q000097:blood* / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D011446:Prospective Studies; D012189:Retrospective Studies; D065227:Transfusion Reaction; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000628:therapy
chemical_list: D007501:Iron