Virus-associated haemophagocytic syndrome responsive to steroid pulse therapy and double filtration plasmapheresis.
We present an adult patient with haemophagocytic syndrome (HPS) successfully treated with a combination of steroid pulse therapy and double filtration plasmapheresis (DFPP). A 58-year-old male was admitted with high fever, severe renal dysfunction, liver dysfunction and an increased level of lactate dehydrogenase. A serological test for Epstein-Barr (EB) virus showed an elevation of EBNA-IgM antibody titre. There were increased haemophagocytic histiocytes in the bone marrow in addition to thrombocytopenia and disseminated intravascular coagulation (DIC) accompanied by organ dysfunction. EB virus associated haemophagocytic syndrome was diagnosed. On admission, interferon (IFN)-gamma, interleukin (IL)-6, IL-8, granulocyte colony-stimulating factor (G-CSF) and macrophage (M)-CSF were elevated, and were promptly normalized after steroid pulse therapy was initiated. G-CSF and M-CSF gradually decreased after DFPPs was started. To control hypercytokinaemia until treatment for the underlying disease is initiated, steroid pulse therapy and double filtration plasmapheresis are useful.
Department of Internal Medicine, Hyogo Prefectural Awaji Hospital, Sumoto, Hyogo, Japan. kodama@awaji-hosp.sumoto.hyogo.jp
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Kodama
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Kuno
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Koide
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Matsuo
D000914:Antibodies, Viral / Q000097:blood; D016207:Cytokines / Q000097:blood; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D019309:Epstein-Barr Virus Nuclear Antigens / Q000097:blood / Q000276:immunology; D005374:Filtration; D015616:Histiocytosis, Non-Langerhans-Cell / Q000628:therapy* / Q000821:virology; D006801:Humans; D008107:Liver Diseases / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D020551:Pulse Therapy, Drug; D051437:Renal Insufficiency / Q000628:therapy; D013256:Steroids / Q000008:administration & dosage*; D013921:Thrombocytopenia / Q000209:etiology
D000914:Antibodies, Viral; D016207:Cytokines; D019309:Epstein-Barr Virus Nuclear Antigens; D013256:Steroids