title: Macrophage activation syndrome in a child with systemic juvenile rheumatoid arthritis.
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.
authors.affiliation: Department of Laboratory Medicine, Hallym University College of Medicine, Seoul, Korea.
authors.forename: Mina
authors.initials: M
authors.lastname: Hur
authors.forename: Young Chul
authors.initials: YC
authors.lastname: Kim
authors.forename: Kyu Man
authors.initials: KM
authors.lastname: Lee
authors.forename: Kwang Nam
authors.initials: KN
authors.lastname: Kim
mesh_terms: D000410:Alanine Transaminase / Q000378:metabolism; D000469:Alkaline Phosphatase / Q000378:metabolism; D015703:Antigens, CD / Q000097:blood; D015214:Antigens, Differentiation, Myelomonocytic / Q000097:blood; D001171:Arthritis, Juvenile / Q000097:blood / Q000150:complications* / Q000473:pathology; D001219:Aspartate Aminotransferases / Q000378:metabolism; D001772:Blood Cell Count; D006529:Hepatomegaly / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D008099:Liver / Q000201:enzymology / Q000473:pathology; D008262:Macrophage Activation; D008297:Male; D010314:Partial Thromboplastin Time; D011517:Prothrombin Time; D013163:Splenomegaly / Q000209:etiology* / Q000473:pathology; D013577:Syndrome; D005723:gamma-Glutamyltransferase / Q000378:metabolism; D000097382:CD68 Molecule
chemical_list: D000410:Alanine Transaminase; D000469:Alkaline Phosphatase; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D001219:Aspartate Aminotransferases; D005723:gamma-Glutamyltransferase; C067980:CD68 antigen, human; D000097382:CD68 Molecule