Table 1.

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.

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