SUCCESSFUL TREATMENT WITH CYCLOSPORINE IN A CASE OF HEMOPHAGOCYTIC SYNDROME MANIFESTING AS SEVERE LIVER DYSFUNCTION

Forms of hemophagocytic syndrome, which affects mainly children, vary from mild to very severe and often fatal, We describe an adult patient with hemophagocytic syndrome in whom severe liver dysfunction develop ed, The condition continued to deteriorate despite treatment with plas ma exchange, high-dose gamma globulin, and corticosteroid therapy, Tre atment with cyclosporine (2.3.mg/kg/day) dramatically improved the con dition and normalized liver function, Cyclosporine reduced the serum l evels of ferritin, interferon-tau, interleukin-6, and soluble interleu kin-a receptor, These findings suggest that hemophagocytic syndrome ac companied with severe liver dysfunction results from hypercytokinemia, and cyclosporine is useful in preventing a fatal outcome during the a cute phase.