Table II.

HLH diagnostic criteria fulfilled by the complete AR STAT1 LOF patient (according to HLH-2004^15,19)

Criterion	Found in patient?
Fever	Yes
Splenomegaly	No
Bicytopenia	Yes: hemoglobin, 5.9 g/dL (normal, 10.7-13.4 g/dL), platelets, 8 G/L (normal, 195-464 G/L)
Hypertriglyceridemia or hypofibrinogenemia	Yes: fibrinogen <70 mg/dL (normal, 160-400 mg/dL)
Hemophagocytosis	Yes: HLH cells in bone marrow
Elevated ferritin	Yes: 22,935 ng/mL (normal, 12-510 ng/mL)
Low/absent NK-cell activity	No
Elevated soluble CD25 (sIL-2RA)	Yes: 15,718 kU/L (normal, 156-623 kU/L)

NK, Natural killer.