Virus-associated haemophagocytic syndrome responsive to steroid pulse therapy and double filtration plasmapheresis

We present an adult patient with haemophagocytic syndrome (HPS) successfull y treated with a combination of steroid pulse therapy and double filtration plasmapheresis (DFPP). A 58-year-old male was admitted with high fever, se vere renal dysfunction, liver dysfunction and an increased level of lactate dehydrogenase. A serological test for Epstein-Barr (EB) virus showed an el evation of EBNA-IgM antibody titre. There were increased haemophagocytic hi stiocytes in the bone marrow in addition to thrombocytopenia and disseminat ed intravascular coagulation (DIC) accompanied by organ dysfunction. EB vir us associated haemophagocytic syndrome was diagnosed. On admission, interfe ron (IFN)-gamma, interleukin (IL)-6, IL-8, granulocyte colony-stimulating f actor (G-CSF) and macrophage (M)-CSF were elevated, and were promptly norma lized after steroid pulse therapy was initiated. G-CSF and M-CSF gradually decreased after DFPPs was started. To control hypercytokinaemia until treat ment for the underlying disease is initiated, steroid pulse therapy and dou ble filtration plasmapheresis are useful.