Interpretation
| Result | Meaning |
|---|---|
| ≤ 90 | < 1% probability |
| 130 | ≈ 9% |
| 169 (best cutoff) | ≈ 54%; sens 93%, spec 86% |
| 200 | ≈ 88% |
| ≥ 250 | > 99% |
Formula
Immunosuppression 18 · Temp <38.4 0 / 38.4–39.4 33 / >39.4 49 · Organomegaly none 0 / liver or spleen 23 / both 38 · Cytopenias (Hb ≤9.2 g/dL, WBC ≤5 ×10⁹/L, plt ≤110 ×10⁹/L): 1 lineage 0 / 2 24 / 3 34 · Ferritin <2000 0 / 2000–6000 35 / >6000 50 ng/mL · TG <1.5 0 / 1.5–4 44 / >4 64 mmol/L · Fibrinogen >2.5 0 / ≤2.5 30 g/L · AST <30 0 / ≥30 19 U/L · Marrow hemophagocytosis 35. Probability = linear interpolation of Fardet 2014 Table 5 (published every 10 points).
Clinical pearls
- Designed for adults with suspected reactive (secondary) HLH; in children HLH-2004 criteria remain standard.
- Bone marrow hemophagocytosis is optional; the derivation study scored missing data as 0, so the score can be calculated before a marrow is done.
- Use the worst values during the episode. Triglycerides: 1.5 mmol/L ≈ 133 mg/dL, 4 mmol/L ≈ 354 mg/dL.
Examples
hscore temp 39.6, hepatosplenomegaly, hb 8, wbc 2.1, plt 60, ferritin 12000, tg 3.5 mmol/L, fibrinogen 1.8, ast 140, no immunosuppressionis this hlh: fever 39, splenomegaly, ferritin 4500, trigs 2.2, fib 2.0, ast 85, hb 9, wbc 3.5, plt 150h score for macrophage activation syndrome
FAQ
What HScore suggests HLH?
The best cutoff is 169 points (93% sensitivity, 86% specificity); probability exceeds 99% at 250 or more.
HScore or HLH-2004 criteria?
HScore was developed for reactive HLH in adults and gives a probability; HLH-2004 criteria (5 of 8) came from pediatric familial HLH trials and need sIL-2R and NK activity.