Diagnostic criteria fulfilled by the 369 eligible patients in HLH-2004
| . | n (%) . | ||
|---|---|---|---|
| Yes* . | No* . | Missing/not done* . | |
| Fever | 335 (95) | 16 (5) | 18 (5) |
| Splenomegaly | 314 (89) | 37 (11) | 18 (5) |
| Bicytopenia (≥2/3 cell lineages)† | 323 (92) | 28 (8) | 18 (5) |
| Hypertriglyceridemia or Hypofibrinogenemia‡ | 314 (90) | 33 (10) | 22 (6) |
| Hypertriglyceridemia‡ | 221 (65) | 118 (35) | 30 (8) |
| Hypofibrinogenemia‡ | 257 (75) | 84 (25) | 28 (8) |
| Hemophagocytosis | 287 (82) | 62 (18) | 20 (5) |
| Ferritin§ | 327 (94) | 21 (6) | 21 (6) |
| Low/absent NK-cell activity|| | 171 (71) | 69 (29) | 129 (35) |
| Soluble CD25 (sIL-2r)¶ | 216 (97) | 7 (3) | 146 (40) |
| . | n (%) . | ||
|---|---|---|---|
| Yes* . | No* . | Missing/not done* . | |
| Fever | 335 (95) | 16 (5) | 18 (5) |
| Splenomegaly | 314 (89) | 37 (11) | 18 (5) |
| Bicytopenia (≥2/3 cell lineages)† | 323 (92) | 28 (8) | 18 (5) |
| Hypertriglyceridemia or Hypofibrinogenemia‡ | 314 (90) | 33 (10) | 22 (6) |
| Hypertriglyceridemia‡ | 221 (65) | 118 (35) | 30 (8) |
| Hypofibrinogenemia‡ | 257 (75) | 84 (25) | 28 (8) |
| Hemophagocytosis | 287 (82) | 62 (18) | 20 (5) |
| Ferritin§ | 327 (94) | 21 (6) | 21 (6) |
| Low/absent NK-cell activity|| | 171 (71) | 69 (29) | 129 (35) |
| Soluble CD25 (sIL-2r)¶ | 216 (97) | 7 (3) | 146 (40) |
Displayed are patients (n) who fulfilled each criterion. For the yes/no columns, the percentage is given without including patients with missing data or for whom the analysis was not done. For the missing/not done columns, the percentage is given for the whole population.
Hb, <90 g/L; platelets, <100 × 109/L; ANC, <1.0 × 109/L.
Fasting triglycerides, ≥3.0 mmol/L (265 mg/dL), fibrinogen, ≤1.5 g/L.
Ferritin, ≥500 µg/L.
Low/absent (<10 lytic units) NK-cell activity.
Soluble CD25 (sIL-2r), ≥2400 U/mL.