Children's Hospital of Philadelphia/University of Pennsylvania Philadelphia, Pennsylvania, United States
Background/Case Studies: Fat embolism syndrome (FES) is a potential devastating complication of sickle cell disease (SCD). The release of fat globules and other by products of bone marrow necrosis into the circulation results in an embolic phenomenon with both mechanical obstruction and inflammation, resulting in stroke and pulmonary embolism symptoms. FES has high morbidity and mortality. Retrospective analysis suggests that red blood cell (RBC) exchange reduces mortality. Recently, several reports have also suggested that the addition of plasma exchange may also be beneficial.
Study
Design/Methods: Here, we report the excellent outcome of a pediatric SCD patient with genotype Sβ+ who developed encephalopathy secondary to FES in the setting of acute parvovirus infection managed with combined RBC and plasma exchanges.
Results/Findings: The patient is a 15-year-old female with sickle cell disease (SCD), genotype Sβ+ thalassemia without a history of significant SCD-related complications. She initially presented with leg and back pain and lower extremity paresthesias. Her workup was notable for a decrease in her hemoglobin and reticulocyte count. Parvovirus PCR was found to be positive. She was admitted for management of a vaso-occlusive pain episode and received one unit of packed RBCs for aplastic crisis. She subsequently developed fever and oxygen desaturations and was started on antibiotics for management of acute chest syndrome. The next day, she became increasingly somnolent with worsening hypoxemia and had progressive decline in her mental status. Head imaging was obtained and showed findings consistent with cerebral fat emboli, confirming the diagnosis of fat embolism syndrome. Imaging of the chest also showed findings consistent with microscopic pulmonary fat emboli. She underwent emergent red blood cell exchange to decrease hemoglobin S percentage and to remove lipids and hyperinflammatory cytokines. During the procedure, it was noted that there was a thick lipid layer in the waste bag of the red cell exchange lying above the red cell layer and below a thin layer of normal appearing plasma (figure 1). Her triglyceride and ferritin levels were found to be significantly elevated. At that time, plasma exchange with fresh frozen plasma was initiated. After three daily plasma exchange procedures, she started to show neurologic improvement and did not display any evidence of end organ damage to the heart, liver or kidneys. She continued to show significant neurologic recovery and by day 10 of admission, was able to be transferred to inpatient rehabilitation to continue occupational, physical and speech therapies. Since then, she has had full return to baseline neurologic function.
Conclusions: Here we detail successful treatment of a pediatric patient with sickle cell disease with fat embolism syndrome with both red cell and plasma exchange procedures.
