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HScore for haemophagocytic syndrome

Also called HScore, HLH score, reactive haemophagocytic syndrome score, MAS HScore

Weighted score (0–337) estimating the probability of reactive (secondary) haemophagocytic lymphohistiocytosis in adults.

Maximum temperature
Organomegaly
Cytopenias (Hb ≤9.2 g/dL, WBC ≤5 ×10⁹/L, platelets ≤110 ×10⁹/L)
Ferritin (ng/mL)
Triglycerides (mg/dL)
Fibrinogen (mg/dL)
AST / SGOT (IU/L)

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Formula and scoring

Immunosuppression 18; temperature 33/49; organomegaly 23/38; cytopenias 24/34; ferritin 35/50; triglycerides 44/64; fibrinogen ≤250 mg/dL 30; AST ≥30 IU/L 19; marrow haemophagocytosis 35. Maximum 337.

Exam and ward pearls

  • Probability <1% at HScore ≤90 and >99% at ≥250 (Fardet 2014).
  • Cut-off 169 is widely quoted (sensitivity 93%, specificity 86%); in adult Still's disease a Japanese study found 192 optimal for macrophage activation syndrome.
  • Score it without marrow (0 points) if no aspirate yet; the result can only rise once marrow is examined.

When not to rely on it

  • Derived in a French multicentre retrospective cohort of adults assessed for reactive haemophagocytic syndrome; not validated for primary (familial) HLH.
  • Point values and the 169 cut-off are taken from two independent open-access papers that reproduce the original table; the authors' probability curve could not be verified, so only the published end-points are shown.

Source

  1. Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-20. doi:10.1002/art.38690 · PMID 24782338
  2. Shiga T, Nozaki Y, Tsuchimoto S, Yamazawa H, Ashida C, Tomita D, et al. Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study. Rheumatology (Oxford). 2026;65(9). doi:10.1093/rheumatology/keag473 · PMID 42671130
  3. Ionescu MD, Prajescu B, Taras R, Popescu N, Vidlescu R, Smarandoiu M, et al. Diagnostic challenges in hemophagocytic lymphohistiocytosis, a rare, potentially fatal disease: two case studies. J Clin Med. 2024;13(6):1643 (reproduces the HScore point table). doi:10.3390/jcm13061643 · PMID 38541872

Formula and cut-offs checked against these sources on 8 Nov 2026. Educational tool for clinicians; it does not replace clinical judgement.

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