ABSTRACT

Haemophagocytic syndrome (HS) is a life-threatening systemic disease that can be fatal if treatment is delayed. HS can occur either primarily or secondary to other systemic diseases. A 16-year old female, who had been followed because of juvenile idiopathic arthritis (JIA), was admitted to our hospital with joint pain, joint swelling, difficulty in moving the joints, malaise, anorexia, and fever that continued for 3 days. Skin eruptions appeared on the 5th day of onset of fever. Her laboratory findings were as follows: white blood cells: 5,800 /mm3; platelets: 146,000 /mm3; C-reactive peptide: 129 mg/L, erythrocyte sedimentation rate: 17 mm/h; ferritin: >2,000 ng/mL; lactate dehydrogenase: 403 U/L; triglycerides: 127 mg/dL; and fibrinogen: 289 mg/dL. The diagnosis of macrophage activation syndrome secondary to JIA was established based on fever lasting longer than 7 days, presence of bicytopenia, elevated laboratory findings (particularly ferritin: >2,000 ng/mL and triglycerides), presence of hemophagocytosis in the bone marrow, and increased liver and spleen size. Although high-dose steroid and cyclosporin treatment was given, plasma exchange was also performed because of persisting fever, bicytopenia, and hepatosplenomegaly as well as increased ferritin and LDH levels of >2,000 ng/mL and 1,372 U/L, respectively. JIA is a major cause of secondary haemophagocytic syndrome. This suggests that clinicians should be aware of the development of macrophage activation syndrome if fever persists in children with JIA. Treatment for this life-threatening disease must be started immediately.

Keywords: Macrophage activation syndrome, juvenile idiopathic arthritis, children