Abstract：
:Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
journal_name
J Korean Med Scijournal_title
Journal of Korean medical scienceauthors
Hur M,Kim YC,Lee KM,Kim KNdoi
10.3346/jkms.2005.20.4.695keywords:
subject
Has Abstractpub_date
2005-08-01 00:00:00pages
695-8issue
4eissn
1011-8934issn
1598-6357pii
200508695journal_volume
20pub_type
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