Table 2.

HLH diagnostic criteria.

Our patient	HLH criteria
Axillary temperature = 38.5°C	Fever (defined as a temperature >100.3 F, >38°C)	✓
Splenomegaly	Enlargement of the spleen	✓
Bicytopenia: Hb = 7.9 mg/dl; WBC = 2300/μl	Decreased blood cell counts affecting at least two of three lineages in the peripheral blood	✓
Hypertriglyceridemia: 2316 mg/dl	High blood levels of triglycerides (fasting, greater than or equal to 265 mg/100 ml) and/or decreased amounts of fibrinogen in the blood (≤150 mg/100 ml)	✓
Increased ferritin: >800 ng/ml	Ferritin ≥500 ng/ml	✓
None	Hemophagocytes in the bone marrow, spleen, or lymph nodes	✘
Not checked	Low or absent natural killer cells' activity	✘
Not checked	Soluble CD25 (soluble IL-2 receptor) > 2400 U/ml (or per local reference laboratory)	✘