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

Fat embolism syndrome in sickle cell disease is associated with great mortality, while more than half of survivors suffer severe neurological sequelae. Release of fat droplets leads to obstruction of the microcirculation as well as generation of proinflammatory cytokines that can cause direct tissue injury. Red cell exchange transfusion can be life-saving but the addition of therapeutic plasma exchange may further improve outcomes by removing such inflammatory mediators.