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

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

Design: Retrospective chart review.

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

Results: 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.

Conclusions: 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.

Implications For Practice: 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.