To outline a rare case of cerebral fat embolism syndrome (FES), from bone marrow necrosis during a sickle cell-thalassemia crisis, that was successfully treated with erythrocytapheresis. 

27 year old woman with heterozygous HbS-beta thalassemia disease and prior history of acute chest syndrome was found down at home unresponsive. On exam, she was obtunded with intact brain stem reflexes and symmetric withdrawal. Lab work suggested active hemolysis and peripheral smear showed numerous nucleated RBCs. Toxicology and infectious work-up was unrevealing including lumbar puncture. EEG showed generalized slowing and initial MRI brain revealed restricted diffusion symmetrically in deep grey matter. Repeat MRI brain performed 3 days later due to persistent coma showed widespread micro-infarcts and micro-hemorrhages in a characteristic “starfield” pattern. CT shoulder showed right humerus head sclerosis. Despite absence of cutaneous or pulmonary features, her coma with no other explanation along with peripheral smear and imaging pattern, raised concern for isolated cerebral fat embolism. Bone marrow biopsy was performed and she immediately underwent exchange transfusion with rapid improvement in her mental status within 48 hours. Bone marrow biopsy subsequently confirmed avascular osteonecrosis.

Our case is a unique amalgam of many rarities including, isolated cerebral FES without pulmonary or cutaneous features, non-traumatic bone marrow necrosis from hemoglobinopathy crisis as the precipitant for FES and demonstration of erythrocytapheresis as a safe and successful treatment option in FES. This hopefully adds to the growing literature on the benefits of early erythrocytapheresis in hemoglobinopathies, not just in vaso-occusive crisis related strokes, but also in FES from avascular bone necrosis.