J Korean Med Sci.  2005 Aug;20(4):695-698. 10.3346/jkms.2005.20.4.695.

Macrophage Activation Syndrome in a Child with Systemic Juvenile Rheumatoid Arthritis

• Hur M
• Kim YC
• Lee KM
• Kim KN

• KMID: 1712759
• DOI: http://doi.org/10.3346/jkms.2005.20.4.695

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.

Keyword

Macrophages; Arthritis; Juvenile Rheumatoid

MeSH Terms

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

Figure

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  Fig. 1 Swelling of both lower legs and feet.

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  Fig. 2 Bone marrow biopsy section shows increased numbers of diffusely distributed and minimally clustered macrophages (CD68 immunostain, ×200).

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  Fig. 3 Laboratory findings of liver enzymes during hospital days. AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; GGT, gamma glutamyl transferase; LDH, lactate dehydrogenase; HD, hospital day.

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  Fig. 4 Laboratory findings of PT, aPTT, and fibrinogen during hospital days. PT, prothrombin time; aPTT, activted partial thromboplastin time; HD, hospital day.

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  Fig. 5 Laboratory findings of complete blood cell counts during hospital days. At the third hospital day, red blood cell was transfused. Hb, hemoglobin; WBC, white blood cell; PLT, platelet; HD, hospital day.

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