A case report of the effect of plasma exchange on reactive hemophagocytic syndrome associated with toxic shock syndrome

We report here the case of a patient suffering from hemophagocytic syndrome (HPS) associated with toxic shock syndrome (TSS). A 50-year-old man was admitted because of fever, watery diarrhea and shortness of breath. Clinical analysis revealed systemic cyanosis, sunburn-like erythema and septic shock. Staphylococcus aureus was identified from both blood and sputum culture and the serum enterotoxin A antibody test was positive, suggesting that this was a case of TSS. Though the respiratory and hemodynamic status improved by the mechanical ventilation, fluid resuscitation with catecholamine and antibiotic therapy, the platelet count decreased rapidly. Bone marrow aspiration revealed a large quantity of hemophagocytosis by macrophages. This reactive HPS was treated not with immunosuppressive drugs but with therapeutic plasma exchange in order to prevent worsening of S. aureus infection. After plasma exchange, the circulating macrophage colony-stimulating factor (M-CSF) level was reduced and the platelet count increased rapidly. Bacteria associated HPS remains a difficult diagnosis with high mortality and there is a crucial question of whether this should be treated with immunosuppressive drugs. The patient's clinical course would suggest that the therapeutic plasma exchange should be considered as a therapeutic tool for the bacteria associated HPS instead of immunosuppressive drugs.