RESUMO
A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
