Table 2.
HLH criteria met in the patient.
| Criteria | Presence | Highest/lowest value | Reference range |
|---|---|---|---|
| Fever | Yes | ||
| Cytopenias | Yes | Hb 7.9 g/dL | 11.7–15.3 |
| Thrombocytes 48 × 109/L | 165–387 | ||
| WBC 0.9 × 109/L | 3.5–10 | ||
| Splenomegaly | Yes | ||
| Hypertriglyceridemia and/or hypofibrinogenemia | Yes | Triglycerides 318 mg/dL | <260 mg/dL |
| Fibrinogen 2.8 g/L | 1.9–4.0 g/L | ||
| Hyperferritinemia | Yes | 29,587 μg/L | 15–200 μg/L |
| Hemophagocytosis in bone marrow | Yes | ||
| Elevated s-IL2 R | Yes | 3,209 IU/L | 160–620 IU/mL |
| Reduced/absent NK-cell activity | No |
The patient met a total of seven out of eight criteria during the course of disease.