Non-Traumatic Fat Embolism Syndrome in Sickle Cell Disease Complicated by Bone Marrow Necrosis Successfully Treated with Therapeutic Plasma Exchange: A Case Report.

BACKGROUND

Non-traumatic fat embolism syndrome (NT-FES) is a rare but frequently fatal complication of sickle cell disease (SCD) that is often associated with bone marrow necrosis. A delayed diagnosis or lack of standard criteria leads to a higher mortality rate. Firstline therapy in NT-FES is red cell exchange transfusion (RCE); however, therapeutic plasma exchange (TPE) is considered to be an adjunct or potential alternative in recent times.

CASE PRESENTATION: We report a case of a middle-aged male with HbSS sickle cell disease and α-thalassemia trait who presented with a vaso-occlusive crisis, which led to severe multiorgan failure, including acute respiratory failure, seizures, acute kidney injury requiring continuous renal replacement therapy, acute hepatic injury, and cardiac ischemia. The laboratory reports showed remarkable hemolysis, hyperferritinemia and cytopenia. The clinical symptoms were consistent with NT-FES secondary to bone marrow necrosis.

INTERVENTION: The patient went through three sessions of therapeutic plasma exchange along with supportive therapy due to alloimmunization and unavailability of compatible RCE.

OUTCOME: The patient showed remarkable clinical improvement, which included recovery of consciousness, resolution of seizures, stabilization of hemolysis markers, and normalization of renal and hepatic parameters.

CONCLUSION

This case demonstrates NT-FES as a critical and underrecognized complication of sickle cell disease. Immediate intervention is necessary to avoid mortality; TPE therapy presents itself as a potential life‑saving intervention in cases with immuno-incompatibility and delayed RCE.