Table 3.
HLH-2004 diagnostic criteria (for use in non-pregnant individuals). 54
| HLH-2004 diagnostic criteria (≥5 of 8 criteria below) |
|---|
| 1. Fever |
| 2. Splenomegaly |
| 3. Cytopenia affecting ≥2 of 3 peripheral blood
lineages – Haemoglobin < 90 g/L – Platelets < 100 ×109/L – Neutrophils <1.0 × 109/L |
| 4. Hypertriglyceridaemia and/or hypofibrinogenaemia – Fasting triglycerides ≥3.0 mmol/L – Fibrinogen ≤1.5 g/L |
| 5. Haemophagocytosis in bone marrow, spleen or lymph nodes with no evidence of malignancy |
| 6. Low or no NK cell activity |
| 7. Ferritin ≥500 µg/L |
| 8. Soluble CD25 (i.e. soluble interleukin-2 receptor, sIL-2R) ≥2400 U/ml |