Macrophage activation syndrome (MAS) is a life-threatening syndrome characterized by persistent fever, hepatosplenomegaly, cytopenia, coagulopathy, hypofibrinogenemia, hyperlipidemia, rapid fall in erythrocyte sedimentation rate, high levels of serum ferritin and liver enzymes and infiltration of vital organs by non-Langerhans histiocytes. This syndrome is rapidly fatal without early diagnosis and institution of therapy. The clinical and laboratory findings that occur suddenly are characteristic. In this article, we describe a three-year-old girl with systemic onset juvenile chronic arthritis who developed MAS after blood transfusion.

Keywords: makrofaj aktivasyon sendromu, jüvenil kronik artrit, kan transfüzyonu, macrophage activation syndrome, juvenile chronic arthritis, blood transfusion.