Criteria | At diagnosis | After remission | Reference values |
---|---|---|---|
1. Fever | Yes | No | |
2. Splenomegaly | No | No | |
3. Cytopenias (≥2 lineages) | |||
Hemoglobin (g/L) | 79 | 125 | <90 |
Platelets (×109/L) | 198 | 332 | <100 |
Neutrophils (×109/L) | 0.01 | 2.38 | <1.0 |
4. Hypertriglyceridemia and/or hypofibrinogenemia | |||
Triglycerides (mmol/L) | 1.98 | 0.69 | ≥3.0 |
Fibrinogen (g/L) | 4.2 | 5.3 | ≤1.5 |
5. Hemophagocytosis | Yes (bone marrow) | ||
6. NK cell activity (%) | 1.02 | 19.39 | ≤8 |
7. Ferritin (μg/L) | 1031 | 259 | ≥500 |
8. Soluble CD25 (U/ml) | 8910 | 1341 | ≥2400 |