Table 2 Diagnostic criteria for hemophagocytic syndrome according to trial HLH-2004

Five of eight clinical and laboratory criteria:

1. Fever

2. Splenomegaly

3. Cytopenia affecting two or more cell lines:

• Hemoglobin <9g/dL

• Platelets <100,000/mL

• Neutrophils <1000/mL

4. Hypertriglyceridemia (fasting triglycerides >265mg/dL or 3mMol/L) and or hypofibrinogenemia (<150mg/dL)

5. Hemophagocytosis in bone marrow, spleen, liver, lymph node, skin

6. Decreased or absent natural killer cell activity

7. Ferritin >500ng/mL^a

8. Soluble CD25 >2400U/mL