Table 3.
Sensitivity and specificity of fulfilled HLH-2004 criteria and HScore
| Parameters | AUC (95% CI) | Cutoff | Sensitivity/specificity |
|---|---|---|---|
| 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% |
Receiver operating characteristics (ROC) analysis to determine the best prediction accuracy of each diagnostic variable for HLH diagnosis. CD107a testing as a functional marker for identification of NK cell activity not shown as performed in 4 patients only. The predictive values of ferritin and sIL-2R of the cohort were already analyzed in Lachmann et al. [8]
AUC Area under the curve, CI confidence interval