Table 4.
HScore criteria as reported by Fardet et al. 18 and “modified 2004-HLH-diagnostic-criteria”.
| HScore – Sum of points* | Modified 2004 diagnostic criteria * | Points |
|---|---|---|
| Immunosuppression: 18 | n.d | |
| Fever: 0 (strictly < 38.4), 33 (38.4–39.4), or 49 (strictly > 39.4) | Fever ≥ 38.5 °C (if persistent -7d) | + 1 |
| Organomegaly: 0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly) | Splenomegaly | + 1 |
| Cytopenia (lowest within 7d): | Cytopenia | + 1 |
| 0 (one lineage), 24 (two lineages), or 34 (three lineages): | (at least bi-lineage, persistent / progressive within 7d) | |
| Hgb ≤ 92 g/L | Hgb < 90 g/L | |
| Platelets ≤ 110 × 109/L | Platelets < 100 × 109/L | |
| Leucocytes ≤ 5 × 109/L | Neutrophils < 1 × 109/L* | |
| Ferritin: 0 (< 2000 ng/ml), 35 (2000–6000 ng/ml), or 50 (> 6000 ng/ml) | Ferritin ≥ 10,000 ng/ml * | + 1 |
| TAG [mg/dl]: 0 (< 132.7), 44 (132.7–354), or 64 (> 354) | TAG [mg/dl]* > 150 | + 1 |
| Fibrinogen [mg/dl]: 0(> 250) or 30 (≤ 250) | Fibrinogen [mg/dl] * < 200 | + 1 |
| AST ≥ 30 U/l: 19 | n.d | |
| Hemophagocytosis in bone marrow – not assessed (35 points) | Hemophagocytosis in bone marrow – not assessed | n.a |
*We used centre specific cut-offs for definition of hypo-fibrinogenaemia, hypertriglyceridemia, leucopenia and neutropenia with respect to the “modified HLH2004 criteria”. We used lower or upper laboratory specific reference ranges to define the adjusted cut offs. In addition the ferritin criterion was modified within the adjusted 2004 HLH-diagnostic guidelines to a cut off of ≥ 10.000 ng/ml, according to better specificity in ICU-patients 19. The HScore is calculated as a sum of points – see Fardet et al. 18. The sum-score can be transformed into a probability score of HLH – however, this has never been validated for assessment of HLH in an ICU-setting. Abbreviations: Aspartate-aminotransferase (AST), Haemoglobin (Hgb), triglycerides (TAG).