Non-Traumatic Fat Embolism Syndrome in Two HbSS Sickle Cell Disease Patients: A Case Series and Review.

Abstract

Non-traumatic fat embolism syndrome (FES) in sickle cell disease (SCD) is associated with high morbidity and mortality. This severe complication arises from bone marrow necrosis, which subsequently can lead to fat embolism syndrome and multi-organ failure. A key challenge in diagnosing and managing this syndrome lies in the absence of established diagnostic criteria and the variability of its clinical presentation. Instant red cell exchange transfusion could be lifesaving once the syndrome is suspected, and the use of therapeutic plasma exchange, as suggested in the literature, can improve outcomes. Here, we report two cases of SCD with FES that were managed successfully with blood transfusion and plasma exchange. Both of them rapidly deteriorated and required intensive care unit admission, where they received red cell exchange followed by plasma exchange. They both made a good recovery and were discharged from the hospital within two weeks.