Macrophage Activation Syndrome in a Child with Systemic Juvenile Rheumatoid Arthritis.
10.3346/jkms.2005.20.4.695
- Author:
Mina HUR
1
;
Young Chul KIM
;
Kyu Man LEE
;
Kwang Nam KIM
Author Information
1. Department of Laboratory Medicine, Hallym University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Macrophages;
Arthritis;
Juvenile Rheumatoid
- MeSH:
Alanine Transaminase/metabolism;
Alkaline Phosphatase/metabolism;
Antigens, CD/blood;
Antigens, Differentiation, Myelomonocytic/blood;
Arthritis, Juvenile Rheumatoid/blood/*complications/pathology;
Aspartate Aminotransferases/metabolism;
Blood Cell Count;
Hepatomegaly/*etiology/pathology;
Humans;
Infant;
Liver/enzymology/pathology;
*Macrophage Activation;
Male;
Partial Thromboplastin Time;
Prothrombin Time;
Splenomegaly/*etiology/pathology;
Syndrome;
gamma-Glutamyltransferase/metabolism
- From:Journal of Korean Medical Science
2005;20(4):695-698
- CountryRepublic of Korea
- Language:English
-
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.
