Table III.
Comparison of the classification criteria for macrophage activation syndrome 2005 and 2016 with symptoms developed in a reported case 1
| Criterion | 2005 [18] | 2016 [13] | Case 1 |
|---|---|---|---|
| Laboratory criteria | |||
| Ferritin, ng/ml | – | > 684 | 1389 |
| Platelets, cells/µl | ≤ 262,000 | ≤ 180,000 | 176,000 |
| AST, U/l | > 59 | > 48 | 58 |
| Triglycerides, mg/dl | – | > 156 | 139 |
| Fibrinogen, mg/dl | ≤ 250 | ≤ 360 | 313 |
| Leucopenia, cells/µl | ≤ 4,000 | – | 14,600–4,900 |
| Clinical criteria | |||
| Central nervous system dysfunction | Seizures, irritability coma, lethargy | – | Seizures, coma, mood changes |
| Hemorrhages | Purpura, bruising, bleeding | – | – |
| Hepatomegaly | > 3 cm | – | 2 cm |
| Histopathological criteria | Macrophages hemophagocytosis in the bone marrow | – | na |
| Diagnosis | Two or more laboratory criteria; any 2, 3, or more clinical and/or laboratory criteria | A febrile patient with known or suspected JIA plus ferritin, plus any two other laboratory criteria |
AST – aspartate aminotransferase, JIA – juvenile idiopathic arthritis, na – not available.