Table 1.

HLH-2004 criteria at diagnosis

Fever	Yes
Splenomegaly	Yes
Cytopenias (affecting ≥ 2 of 3 lineages)
Haemoglobin < 90 g/L	77
Platelets < 100 × 109/L	75
Neutrophils < 1.0 × 109/L	2.17
Hypertriglyceridemia and/or hypofibrinogenemia
Fasting triglycerides ≥ 3.0 mmol/L	3.07
Fibrinogen ≤ 1.5 g/L	5.4
Hemophagocytosis in bone marrow or spleen or lymph nodes	Yes
Low or absent NK-cell activity	Yes
Ferritin ≥ 500 µg/L	2135
sIL-2 receptor ≥ 2400U/ml	82,606