Parameters

Number of Points (Criteria for Scoring)

Case

Temperature (℃)

0 (< 38.4), 33 (38.4-39.4), or 49 ( > 39.4)

49

Number of Cytopenias

0 (1 Lineage), 24 (2 Lineages), or 34 (3 Lineages)

24

Organomegaly

0 (no), 23 (Hepatomegaly or Splenomegaly), or 38 (Hepatomegaly and Splenomegaly)

0

Ferritin (Normal Range:15-150 g/mL)

0 (< 2,000), 35 (2,000-6,000), or 50 ( > 6,000)

50

Triglycerides (Normal range: 50-150 mg/dl)

0 (< 132.7), 44 (132.7-354), or 64 ( > 354)

64

Fibrinogen (Normal Range: 1.8-4.0 g/L)

0 ( > 2.5) or 30 ( ≤ 2.5)

30

Serum Glutamic Oxaloacetic Transaminase (IU/L)

0 (< 30) or 19 ( ≥ 30)

0

Hemophagocytosis Features on Bone Marrow Aspirate

0 (no) or 35 (yes)

35

Known Under Lying Immunosuppression

0 (no) or 18 (yes)

18

Total H-score

270

Probability of sHLH

> 99%