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When Pain Crisis Turns Lethal: A Case Report of Fat Embolism Syndrome in Sickle Cell Disease
1Department of Internal Medicine, College of Medicine, Imam Abdulrahman Bin Faisal University, King Fahad Hospital of the University, Dammam 31441, Eastern Province, Saudi Arabia.
Abstract:
Fat embolism syndrome (FES) is a rare, life-threatening complication of sickle cell disease (SCD), often underrecognized due to overlap with acute chest syndrome, sepsis, and thrombotic microangiopathies. We report a 37-year-old man with sickle cell disease who presented with an acute vaso-occlusive crisis and rapidly deteriorated with respiratory failure, cardiovascular collapse, and neurological dysfunction. Common etiologies including pulmonary embolism, infection, and stroke were excluded. Laboratory evidence of hemolysis, cytopenias, elevated ferritin, and acute cor pulmonale suggested systemic FES secondary to bone marrow necrosis. The patient underwent urgent red cell exchange transfusion, followed by therapeutic plasma exchange and corticosteroids, resulting in significant clinical and hemodynamic recovery. This case highlights the importance of early recognition and aggressive multimodal therapy in SCD-FES.
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