Adult onset Still's disease with hemophagocytic syndrome and severe liver dysfunction

We report the case of a 55-year-old Japanese woman with adult onset Still's disease ill whom hemophagocytic syndrome and severe liver dysfunction deve loped. High serum levels of Ferritin, macrophage colony stimulating factor and interferon-gamma, which imply the presence of hemophagocytic syndrome; were detected. It is known that hemophagocytic syndrome is associated with adult onset Still's disease. In our case, many markedly swollen Kupffer cel ls with phagocytized red blood cells were found in the liver, as well as ma crophages in the bone marrow and spleen. Accordingly, we believe that sever e liver dysfunction in this case may have been related to hypercytokinemia due to hemophagocytic syndrome. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.