A Case of Fat Embolism Syndrome with Cerebral Involvement in Sickle Cell Anemia

Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion

“…[1][2][3][4][5] It manifests with myriad of clinical signs and symptoms, including hypoxemia, neurologic changes, petechial rash, and multiorgan dysfunction. [1][2][3][4][5][6][7] Upon diagnosis, an immediate blood cell exchange transfusion (RCE) is recommended, the outcome of which remains unsatisfactory with significant mortality and long-term debilitating neurological complications. 1,2,[4][5][6][7] Therefore, calls were made for a more effective therapeutic interventions.…”

“…Vaso‐occlusive crises (VOC) can cause bone infarcts, and the subsequent release of fat globules into the microcirculation leads to FES 1–5 . It manifests with myriad of clinical signs and symptoms, including hypoxemia, neurologic changes, petechial rash, and multi‐organ dysfunction 1–7 . Upon diagnosis, an immediate blood cell exchange transfusion (RCE) is recommended, the outcome of which remains unsatisfactory with significant mortality and long‐term debilitating neurological complications 1,2,4–7 .…”

“…[1][2][3][4][5][6][7] Upon diagnosis, an immediate blood cell exchange transfusion (RCE) is recommended, the outcome of which remains unsatisfactory with significant mortality and long-term debilitating neurological complications. 1,2,[4][5][6][7] Therefore, calls were made for a more effective therapeutic interventions. 1,8 Tsitsikas et al suggested that therapeutic plasma exchange (TPE) may improve the outcome.…”