TABLE 2.

Criteria of hemophagocytic lymphohistiocytosis (HLH) and H‐Score

Clinical and Laboratory Criteria
Clinical criteria	Demonstrated by patient	H‐Score criteria	H‐Score points for patient
Fever	Yes	Fever	49 points (T max 40.5°C)
Splenomegaly	Yes	Organomegaly	38 points (hepatomegaly and splenomegaly)
Cytopenias (2 or 3 lineages) ‐ Hemoglobin (<100 g/L in infants <4 weeks) ‐ Platelets < 100 × 109/L ‐ Neutrophils < 1.0 × 109/L	No Yes No No	Cytopenias	0 points (1 lineage)
Ferritin ≥ 500 mg/L	Yes	Hyperferritinemia	50 points (> 6000 ng/ml)
Hemophagocytosis	Yes	Hemophagocytosis in bone marrow?	35 points (Yes)
Triglycerides ‐ Fasting triglycerides ≥ 3.0 mmol/L ‐ Fibrinogen ≤ 1.5 g/L	Yes Yes No	Triglycerides	64 points (> 4 mmol/L)
Low/absent NK‐cell activity	Yes	N/A
Elevated soluble CD25/IL‐2 receptor (≥ 2400 U/ml)	Yes	N/A
Serum glutamic oxaloacetic transaminase (SGOT)	N/A	SGOT	0 points (Not drawn in this patient)
Total diagnostic criteria (5 of 8 required)	Yes (7 of 8)	H‐Score total	236*
Genetic Criteria
Gene Panel		No

^*98%–99% probability of hemophagocytic syndrome according to H‐Score [13].