Table 3 Sensitivity and specificity of fulfilled HLH-2004 criteria and HScore

HLH-2004 criteria

0.982 (0.971–0.993)

2

100%/38.4%

3

100%/77.8%

4

95.0%/93.6%

5

72.5%/98.7%

6

45.0%/99.9%

7

10.0%/100%

HScore

0.992 (0.987–0.996)

140

100%/88.2%

150

100%/90.7%

160

100%/93.1%

168

100%/94.1%

170

97.5%/94.2%

180

95.0%/95.3%

190

95.0%/96.3%

200

90.0%/97.3%

210

85.0%/98.3%

Bi-/pancytopenia

0.871 (0.822–0.920)

Yes

92.5%/81.8%

Hypofibrinogenemia or hypertriglyceridemia

0.780 (0.702–0.859)

Yes

75.0%/81.0%

 Fibrinogen [g/L]

0.760 (0.677–0.843)

1.5

43.6%/91.1%

3.1

79.5%/64.5%

 Triglycerides [mg/dL]

0.830 (0.776–0.883)

229

84.6%/71.9%

265

66.7%/77.4%

Max. core body temperature [°C]

0.737 (0.656–0.818)

38.5

80.0%/59.8%

Splenomegaly

0.725 (0.638–0.812)

Yes

65.0%/80.0%

Hemophagocytosis

0.724 (0.611–0.837)

Yes

51.6%/93.2%