Further evidence for the benefit of therapeutic plasma exchange for acute multi-organ failure syndrome refractory to red cell exchange in sickle cell disease.

Acute multiorgan failure syndrome (MOFS) remains a significant cause of mortality in sickle cell disease (SCD) patients despite red cell exchange (RCE). In small case series and reports, therapeutic plasma exchange (TPE) has shown benefit in MOFS. As further support for consideration of this modality, we present two patients with SCD and MOFS refractory to RCE who were subsequently treated with TPE. Fresh frozen plasma was used as the replacement fluid. Despite estimated hospital mortality of 40% at the time of intensive care unit admission, both patients showed marked clinical improvement with TPE treatment. Our cases add to the evidence supporting the potential inclusion of MOFS secondary to acute SCD as an indication for TPE in the next edition of the American Society of Apheresis Guidelines on the Use of Therapeutic Apheresis in Clinical Practice.