Objective: To study the pattern, etiology, and significance of renal iron accumulation in chronically transfused sickle cell disease (SCD) and thalassemia major (TM) patients using magnetic resonance imaging (MRI).

Methods: Magnetic resonance imaging (MRI) was performed in 75 SCD patients, 73 TM patients, and 16 healthy controls. Multiecho gradient echo protocols were used to measure T2* reciprocals (R2*) in the kidney, liver, and heart. Kidney R2* was compared to tissue iron estimates, serum iron markers, and surrogates of intravascular hemolysis by univariate regression.

Results: Mean R2* in SCD patients was 55.3+/-45.3 Hz, compared with 22.1+/-11 Hz in TM patients and 21.3+/-5.8 Hz in control subjects (P<0.001). Kidney R2* decreased with advancing age (R2=0.09, P<0.02). Kidney R2* correlated strongly with increased serum lactate dehydrogenase levels found in SCD (R2=0.55, P<0.001), but was independent of hepatic iron concentration and cardiac R2*. Kidney R2* did not correlate with blood pressure, creatinine, cardiac index, or right and left ejection fraction.

Conclusions: Intravascular hemolysis, not chronic transfusion, causes renal hemosiderosis in SCD. Prospective trials are necessary to determine whether kidney R2* is a biomarker for hemolysis-mediated vascular complications in SCD.