HScore for hemophagocytic lymphohistiocytosis
Probability of reactive hemophagocytic syndrome (HLH).
Inputs
CompleteKnown immunosuppression (HIV or long-term immunosuppressive therapy)
Temperature (°C)
Organomegaly
Cytopenias (Hb ≤ 9.2, WBC ≤ 5,000, plt ≤ 110,000)
Ferritin (ng/mL)
Triglycerides
Fibrinogen 250 mg/dL (2.5 g/L) or lower
AST 30 U/L or higher
Hemophagocytosis on bone marrow aspirate
Worked calculation
Your values are highlighted.
- Points
When to use
- Suspected secondary HLH in adults.
Pearls and pitfalls
- Cutoff 169: sensitivity 93%, specificity 86%.
- About 90 or lower: probability under 1%; 250 or higher: above 99%.
Why use
Better suited to adults than the HLH-2004 criteria.
| Criterion | Points |
|---|---|
| Known immunosuppression (HIV or long-term immunosuppressive therapy) | +18 |
| Temperature (°C): Below 38.4 | 0 |
| 38.4–39.4 | +33 |
| Above 39.4 | +49 |
| Organomegaly: None | 0 |
| Hepatomegaly or splenomegaly | +23 |
| Both | +38 |
| Cytopenias (Hb ≤ 9.2, WBC ≤ 5,000, plt ≤ 110,000): One lineage or none | 0 |
| Two lineages | +24 |
| Three lineages | +34 |
| Ferritin (ng/mL): Below 2,000 | 0 |
| 2,000–6,000 | +35 |
| Above 6,000 | +50 |
| Triglycerides: Below 133 mg/dL (1.5 mmol/L) | 0 |
| 133–354 mg/dL | +44 |
| Above 354 mg/dL (4 mmol/L) | +64 |
| Fibrinogen 250 mg/dL (2.5 g/L) or lower | +30 |
| AST 30 U/L or higher | +19 |
| Hemophagocytosis on bone marrow aspirate | +35 |