Hyperferritinämie beim Still-Syndrom des erwachsenen und reaktives hämophgozytisches Syndrom

□ This report describes the fatal outcome of a case of adult onset Still’s disease in a 46-year old man. The diagnosis was made according to the 1992 criteria, proposed by Yamaguchi. Nine months after the initial disease manifestations a rapid deterioration with progressive hepatosplenomegaly developed. In parall, pancytopenia and marked hyperferritinemia could be detected. Tranjugular liver biopsy revealed the presence of a hemophagocytic syndrome. The course of the disease was refractory to any form of treatment and the patient died from disseminated intravascular coagulation, hepatic and pulmonary failure. □ Pathogenetic mechanisms and possible associations between Still’s disease and reactive hemophagocytic syndrome are discussed.