Table 1.
Clinical and laboratory parameters at diagnosis and after remission according to HLH-2004 guidelines
| 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 |