Complete neurological recovery from fat embolism syndrome in sickle cell disease after sequential red cell exchange transfusion and therapeutic plasma exchange

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

“…Since then, similar observations were noted in patients with SCD in whom FES was misdiagnosed as TTP and who were managed with TPE with excellent outcome 1,16 . It is thought that TPE can potentially remove fat droplets and released cytokines from the circulation 9 . Our patient manifested with severe acute FES with thrombotic microangiopathy, which was described previously in SCD patients in the gulf area, 17 and had significant improvement following TPE.…”

“…FES is believed to occur following extensive marrow necrosis resulting in the release of fat globules, free fatty acids, and inflammatory cytokines into the venous and then the systemic circulation 1–6 . Mechanical obstruction, and perhaps more importantly, an inflammatory biochemical process leads to the development of FES with its diverse manifestations 2–5,8,9 . In support of the latter, an interesting case report described FES complicated by cerebral infarction a patient without acute chest syndrome (ACS) or a right to left shunt 11 .…”

“…Our patient manifested with severe acute FES with thrombotic microangiopathy, which was described previously in SCD patients in the gulf area, 17 and had significant improvement following TPE. Tsitsikas et al reported the use of TPE for a variety of indications in SCD 9 . Three patients had FES, 2 recovered uneventfully and one patient with muti‐organ failure died a day following TPE 9 .…”

“…1,8 Tsitsikas et al suggested that therapeutic plasma exchange (TPE) may improve the outcome. 1,9 However, less than a handful of patients managed with TPE were reported. 1,9,10 Herein, we report a patient who had an excellent outcome with TPE after failing to respond to RCE.…”

“…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 1,9 . However, less than a handful of patients managed with TPE were reported 1,9,10 .…”