Blood transfusion and iron overload in patients with Sickle Cell Disease (SCD): Personal experience and a short update of diabetes mellitus occurrence

INTRODUCTION: In patients with sickle cell disease (SCD), vaso occlusive crisis (VOCs) and iron intoxication due to repeated blood transfusion may cause damages in many organs, including the kidneys, lungs and brain, and pancreas. AIM OF OUR STUDY: To evaluate the iron status, hepatic functions and fasting plasma glucose (FPG) in non transfusion dependent (NTD-SCD) patients and transfusion dependent (TD-SCD) patients yearly for 5 years. Cardiac status was evaluated using echocardiography. RESULTS: 16 adults with NT-SCD and six with TD-SCD were studied. 6/16 NTD-SCD had serum ferritin (SF) > 500 ng/mL, and 5/16 patients had high LIC (> 36 μmol Fe/kg dry weight). 2/16 had impaired fasting glucose (IFG). The TD-SCD patients were on top-up transfusion and oral iron chelation therapy (Exjade). All had high LIC, but 2/6 had SF <500 ng/mL. Liver enzymes were high in 2/6 patients. One had IFG. Five years after the initial assessment, 3/16 NT-SCD developed diabetes mellitus (DM) and 2/16 IFG. In TD-SCD, 2/6 developed DM and 1/6 had IFG. Echocardiography revealed abnormalities in 5/22 (22.7%). CONCLUSIONS: A significant number of our patients with ND-SCD and TD-SCD develop dysglycemia, hepatopathy, and echocardiographic abnormalities during the follow-up that need effective early detection and management. However, the prevalence of DM in our SCD remains lower than in the general population in Qatar. TD-SCD patients who developed DM were younger and had high LIC and SF compared to those who developed DM in the NTD-SCD (www.actabiomedica.it).