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5TH NATIONAL CONFERENCE OF PEDIATRIC RHEUMATOLOGY, KOLKATA, 29TH & 30TH SEPTEMBER 2007
Ramesh Bhasi *, Vijayan**, Shalini Kuruvila***, Lilly Rajeevan***
Department of Rheumatology*, Pediatrics** & Pathology***
Malabar Institute of Medical Sciences (MIMS), Calicut, Kerala
Over a period of 5 years 5 children were seen with Macrophage Activation Syndrome. All of them had evidence of significant hemophagocytosis in Bone marrow examination. Two children had underlying SLE and two children had Polyarticular JIA and Systemic Onset - JIA respectively. One child had evidence of sepsis.
High grade fever, cytopenia and elevated transaminases were the common presenting manifestations. In One child MAS was diagnosed 2 weeks after introduction of Methotrexate. Marked elevation of ferritin, low ESR and elevated triglycerides were characteristic, two out of 5 patients survived.
Macrophage activation syndrome is associated with high mortality and should be suspected in children with underlying pediatric rheumatology conditions especially when present with multi system involvement.
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