Clinical, Laboratory and Radiological features, and Outcome of Acute Fat Embolism Syndrome in Sickle Cell Disease

Abstract Non-traumatic fat embolism syndrome (FES) affecting brain, lung and hematopoietic system is a rare, but a serious complication of sickle cell disease (SCD), resulting from bone marrow necrosis. SCD-related FES is rare, with the spectrum of clinical, laboratory, radiological manifestations and patient outcome is not fully understood. After medical research & ethics committee approval, retrospectively, SCD-FES patients at our centre, were reviewed between January 2006 to December 2023. 27 patients (17 males, 10 females) with a median age of 24 years and length of hospital stay of 24 (16–38) days were enrolled. They had fever, chest/back pain, cough and crepitation in 100%, 96%, 56% and 100% respectively, with neurological manifestations in 96%. Abnormal chest X-rays and CT scans were observed in 96%, and 100% respectively. Patients had significant anemia, reticulocytopenia, and thrombocytopenia, with raised WBC (p < 0.05). There was a significant rise in LDH, ALP, Ferritin and C-reactive protein levels. All patients received antibiotics, and exchange transfusions, whereas 24%, 76% required non-invasive ventilation (NIV) and mechanical ventilation respectively, with 18.5% mortality. FES is a rapidly progressive respiratory and neurological syndrome, characterized by hypoxia, and cytopenia, with raised inflammatory markers, raised LDH and ALP, with distinctive multiple cerebral microbleeds.