Table 1. Diagnostic criteria fulfilled by the 20 eligible patients in HLH-2004.
| n = 20 | |
|---|---|
| Fever (≥ 38.5°C) | 20 (100) |
| Splenomegaly | 13 (65) |
| Bicytopenia (2/3 cell lineage) | 15 (75) |
| Hypertriglyceridemia or hypofibrinogenemia | 12 (60) |
| Hypertriglyceridemia ≥ 265 mg/dL | 12 (60) |
| Hypofibrinogenemioa ≤ 150 mg/dL | 4 (20) |
| Hemophagocytosis | 16 (80) |
| Ferritin ≥ 500 ng/mL | 20 (100) |
| Low/absent NK-cell activity | 6 (30) |
| Soluble CD25 ≥ 2400 U/ml | 8 (40) |
| Hscore* > 169 | 20 (100) |
| PRINTO criteria | 20 (100) |
*HScore calculator (for the percentage probability of secondary HLH) is available at http://saintantoine.aphp.fr/score/. HLH: Hemophagocytic lymphohistiocytosis, NK-cell: Natural killer cell, PRINTO: Pediatric Rheumatology International Trials Organization.