Acute severe hepatitis and hemophagocytosis in adult onset Still’s disease

Abstract

We report a 44-year-old male Turkish patient with adult onset Still's disease (AOSD) complicated by acute severe hepatitis and hemophagocytosis. Initial investigations for fever and rapidly progressive elevation of liver function tests were not diagnostic. Routine evaluations of liver and bone marrow biopsies missed the fundamental pathology. Extremely elevated ferritin levels led to a more detailed search, and immunohistochemical staining with CD68 for macrophages revealed extensive hemophagocytosis in both the first and second bone marrow biopsies, as well as in the liver biopsy. Treatment with steroid and cyclosporine A induced complete remission.