Increased Brain Iron Deposition in Patients with Sickle Cell Disease: an MRI Quantitative Susceptibility Mapping Study

Progressive white matter disease, especially silent cerebral infarction (SCI), is a major problem in sickle cell disease (SCD).1,2 Although the risk of symptomatic ischemic stroke has been reduced by chronic transfusion therapy and hydroxyurea, the prevalence of SCI in SCD patients continues to be 1% to 2% per age year with no plateau.2 Many studies have focused on the structural and hemodynamic aspects of SCI,3-5 but few have examined brain iron accumulation6,7 and its possible role in reinforcing white matter injury. Brain iron has been shown to increase with recurrent ischemia-reperfusion injuries,8,9 chronic hypoxia,10,11 and microvasculaturedamage,12 which are common conditions in SCD.13,14 As a consequence, excessive brain iron could potentially aggravate white matter damage15,16 and accelerate neurodegeneration.12