Clinical criteria | ||
2016 MAS classification criteria[1] | 2004 HLH criteria*[2] | |
Diagnostic requirements |
|
|
Fever |
|
|
Organomegaly |
|
|
Cytopenia |
| At least 2 of the following:
|
Miscellaneous laboratory findings |
|
|
Ferritin |
|
|
AST: aspartate aminotransferase; HLA: hereditary lymphohistiocytic hemophagocytosis; MAS: macrophage activation syndrome; N/A: not applicable; sJIA: systemic juvenile idiopathic arthritis; WBC: white blood cell.
* The 2004 HLH diagnostic criteria often cannot be applied to MAS in patients with sJIA because they are too stringent to identify early signs of MAS.