Iron overload in adults with sickle cell disease who have received intermittent red blood cell transfusions

To assess the prevalence of iron overload in adults with sickle cell disease (SCD) not on a chronic transfusion protocol.

Retrospective chart review.

University of South Alabama Comprehensive Sickle Cell Center adult outpatient clinic.

There was no significant difference in units transfused across the four genotypes (HbSS, HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia). Only individuals with HbSS (n = 63) met criteria for iron overload with ferritins of ≥1500 ng/mL. Forty-eight had ferritins <1500 ng/mL, eight (13%) had ferritins ≥3000 ng/mL, and seven (11%) had ferritins ≥1500 and <3000 ng/mL. The overall prevalence of iron overload was 9.74% in SCD cohort and 23.8% in the HbSS genotype.

Our data support that patients with HbSS are at a particularly high risk for inadvertent iron overload as compared to HbSC, HbSβ(0)-thalassemia, and HbSβ(+)-thalassemia.

This study supports the need for healthcare providers to closely monitor the number of red blood cell (RBC) transfusions, RBC units transfused, and serial baseline, steady-state ferritin levels. With closer monitoring, the clinical significance of iron overload in SCD can be established and guide the healthcare provider's management in the prevention of iron overload.