Parvovirus B19-associated fat embolism syndrome in sickle cell disease: A report of two cases

Fat embolism syndrome (FES) is a rare but likely underdiagnosed complication of sickle cell disease (SCD) characterised by multi- or single-organ involvement secondary to embolism of fat and/or necrotic bone marrow. It is one of the most devastating acute complications of SCD and historically the diagnosis has often been made on the basis of post-mortem findings. Literature on FES in SCD is scarce, although most reported cases are individuals with non-HbSS genotypes for reasons which have not yet been fully elucidated. Even fewer data exist regarding the potential association with parvovirus B19 (B19V) and the mechanisms by which B19V infection could trigger FES in SCD. Management is largely supportive, with early intensive care team involvement for close monitoring and potential organ support. Emerging evidence, albeit limited, suggests that early exchange transfusion has an important role in improving clinical outcomes. We describe two life-threatening cases of FES in patients with SCD of differing genotypes, who required intensive care unit admission but who both recovered with supportive management and early exchange transfusion.