[Adult-onset Still disease presenting with disseminated intravascular coagulation]

A 17-year-old female was admitted to our hospital because of pyrexia and thrombocytopenia in May 2002. Laboratory examination showed a platelet count of 50,000/microliter with an increased level of fibrinogen degradation product, leading to a diagnosis of disseminated intravascular coagulation (DIC). Gabexate mesilate was intravenously administrated without any effects. Several days later, erythema, joint pain and neck lymphadenopathy developed sequentially. The patient was diagnosed as having adult-onset Still disease (AOSD) complicated with DIC. Moreover, serum inflammatory cytokine levels had increased and activated macrophages were observed in the bone marrow, suggesting the presence of macrophage activation syndrome. After additional treatments with dalteparin and aspirin, the clinical symptoms and laboratory findings associated with AOSD and DIC disappeared. Although this was a severe case of AOSD associated with preceding DIC, the AOSD symptoms resolved in this patient with the treatment of the DIC and with aspirin only without any relapse.