Background: Kawasaki disease is an acute febrile vasculitis of childhood. Macrophage activation syndrome is a rare life threatening complication.
Case Characteristics: 4-year-old boy with Kawasaki Disease treated with intravenous immunoglobulins.
Observation: He developed encephalopathy, hepatosplenomegaly and pancytopenia. Blood investigations and bone marrow aspiration suggested macrophage activation syndrome.
Outcome: Good response to pulse methylprednisolone (30 mg/kg/d) for 5 days.
Message: Macrophage activation syndrome may complicate Kawasaki disease.
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