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Macrophage with phagocytated platelets and lipids. Giemsa 1000X 

Macrophage with phagocytated platelets and lipids. Giemsa 1000X 

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Background: Most frequently seen in Systemic Arthritis – Juvenile Idiopathic Arthritis, macro-phage activation syndrome (MAS) may also be a life-threatening complication in juvenile systemic lupus erythematosus (SLE). The diagnosis of MAS may be particularly challenging because it may mimic the clinical and laboratory features of the underlying dis...

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Macrophage activation syndrome (MAS) is a fatal complication in rheumatic diseases. It is characterized by prolonged fever, pancytopenia, and hepatosplenomegaly, which are consequences of uncontrolled macrophage activation. MAS in children is most commonly associated with systemic juvenile idiopathic arthritis. Its association with systemic lupus erythematosus (SLE) is relatively rare, so we report a Thai boy who initially presented with MAS and eventually was diagnosed as having SLE. He also had recurrent MAS during the course of therapy. Hyperferritinemia is one of the abnormal laboratory findings in MAS and it has been used as an inflammatory marker. However, its correlation with disease activity remains unclear. Therefore, a review of literature regarding MAS-associated SLE in children and ferritin level in this disease was carried out.