| Clinical criteria |
2004 HLH criteria[1] | 2004 MAS criteria[2] | 2016 MAS classification criteria[3] |
Diagnostic requirements | | - Requires 2 laboratory or >3 clinical and/or laboratory criteria in addition to fever in a patient with systemic JIA
| - Requires ferritin criterion and any other 2 criteria in addition to fever in a patient with systemic JIA
|
Fever | | | |
Organomegaly | | | |
Cytopenia | - 2 of 3 cell lines must be affected
| - Platelet count ≤262,000/microL OR
- WBC ≤4000 cells/microL
| - Platelet count <181,000/microL
|
Miscellaneous laboratory findings | - Triglycerides >265 mg/dL
- OR
- Fibrinogen <1.5 g/L
| | - AST >48 units/L,
- Fibrinogen <360 mg/dL, OR
- Triglycerides >156 mg/dL
|
Ferritin | | | |
Other clinical features | | - CNS dysfunction
- Hemorrhages
| |