Cerebral Fat Embolism Syndrome in Homozygous Sickle Cell Disease Treated With Therapeutic Plasma Exchange and Simple Transfusions - PubMed
6 hours ago
- #Therapeutic Plasma Exchange
- #Sickle Cell Disease
- #Fat Embolism Syndrome
- Cerebral Fat Embolism Syndrome (FES) is a rare, life-threatening complication of sickle cell disease (SCD).
- A 17-year-old male with homozygous SCD (HbSS) developed cerebral FES following vaso-occlusive crisis and acute chest syndrome (ACS).
- Diagnosis was supported by clinical findings and characteristic punctate lesions on brain MRI.
- Treatment included multiple simple red blood cell transfusions and four sessions of therapeutic plasma exchange (TPE), followed by chronic transfusions for one year.
- The patient showed rapid hematologic improvement and full neurologic recovery.
- Only six cases of cerebral FES in HbSS individuals have been reported, mostly in young males.
- Early combined transfusion and TPE appears to yield the most favorable outcomes.
- The case highlights diagnostic challenges and supports early multimodal therapy for cerebral FES in SCD.
- Multicenter studies are needed to define optimal management strategies.