Accelerated brain atrophy in sickle cell anemia despite chronic exchange transfusion therapy-A case report

Neurovascular complications are a common and major cause of morbidity and mortality in patients with sickle cell anemia (SCA). Prior studies have demonstrated cortical thinning as well as reduced cerebral volume in children with SCA when compared to age-matched controls, but data in adults are scarce. Chronic transfusion regimens have been shown to be neuroprotective in children with SCA, but their efficacy in adults is less clear. Automated erythrocytapheresis (red blood cell exchange transfusion) is the most aggressive disease modifying-treatment in SCA, by rapidly diluting sickle hemoglobin and replacing it with normal hemoglobin. Recently, 7 Tesla (7T) MRI have allowed for precise definition and quantitation of brain structures, specifically the delineation between gray and white matter, thereby facilitating the assessment of neurodegeneration in SCA. We hereby present a case of a woman in her late 30s (at the baseline) who was monitored for a period of 6 years (baseline + 2 time points) with 7T structural imaging. The patient has SCA and a remote history of right hemispheric stroke. Despite adherence to a chronic exchange transfusion program, the patient developed accelerated gray matter with a volume loss of 1.15% per year, on average, during the 6-year period. Our case report underscores the severity of accelerated brain atrophy in SCA.