• A set of weighted criteria, producing a score out of 337
  • Score ≥169 commonly used as a cut-off as a likelihood for a diagnosis of HLH
  • Produces a % risk of HLH based upon weighted score
No. of Points
Parameter
No. of Points
(Criteria for scoring)
Known Underlying Immunosuppression

(HIV or Immunosuppressive therapy)

0 (no)
18 (yes)
Fever (C)
0 (<38.4)
33 (38.4-39.4)
49 (>39.4)
Organomegaly
0 (no)
23 (hepatomegaly or splenomegaly)
38 (hepatomegaly and splenomegaly)
Number of Cytopenia

(Hb <9.2 g/L / WBC ≤5 x 109/L / plat ≤110 x 109/L)

0 (one lineage)
24 (two lineages)
34 (three lineages)
Triglycerides (mmol/L)
0 (<1.5)
44 (1.5-4)
64 (>4)
Ferritin (µg/L)
0 (<2000)
35 (2000-6000)
50 (>6000)