Cerebral Fat Embolism Syndrome in Homozygous Sickle Cell Disease Treated With Therapeutic Plasma Exchange and Simple...

Fat embolism syndrome (FES) is a rare, life-threatening complication of sickle cell disease (SCD) associated with high mortality and neurologic morbidity. We report a 17-year-old male with homozygous SCD (HbSS) who developed cerebral FES following vaso-occlusive and acute chest syndrome (ACS). Diagnosis was supported by clinical findings and characteristic punctate lesions on brain MRI. He received multiple simple red blood cell transfusions and four sessions of therapeutic plasma exchange (TPE), followed by 1 year of chronic transfusions. ADAMTS13 activity was mildly reduced but inconsistent with thrombotic thrombocytopenic purpura. The patient experienced rapid hematologic improvement and full neurologic recovery. Including this case, only six reports describe cerebral FES in HbSS individuals, predominantly young males. Outcomes appear most favorable with early combined transfusion and TPE. This case highlights the diagnostic challenges and supports early multimodal therapy for cerebral FES in SCD while underscoring the need for multicenter studies to define optimal management.