From: Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour
(1) Fever | Manifestations in the patient described quotidian fever over several weeks |
---|---|
(2) Splenomegaly | hepatosplenomegaly |
(3) Cytopenia involving two or more cell lines | haemoglobin < 9.0 d/dL, no other |
(4) Hypertriglyceridaemia or hypofibrinogenaemia | no |
(5) Haemophagocytosis | yes |
(6) Hepatitis | yes |
(7) Low or absent natural killer cell activity | yes |
(8) Serum ferritin level > 500 μg | 1043 μg/L |
(9) Soluble CD25 (sIL-2 receptor) > 2500 U/ml | > 7200 U/ml |