Table 2 Diagnostic Criteria of Hemophagocytic Lymphohistiocytosis (HLH) [17]
Molecular diagnosis of HLH or the presence of at least 5 of 8 criteria: | |
|---|---|
1. Fever | |
2. Splenomegaly | |
3. Cytopenia (affecting at least 2 lineages in the peripheral blood), Hemoglobin levels < 90 g/L (in infants < 4 weeks old, hemoglobin < 100 g/L), Platelets < 100 × 109 /L, Neutrophils < 1.0 × 109 /L | |
4. Hypertriglyceridemia and/or hypofibrinogenemia: Fasting triglycerides ≥3.0 mmol/L (ie, ≥ 265 mg/dl), Fibrinogen ≤1.5 g/L | |
5. Documented hemophagocytosis in the bone marrow, spleen, or lymph nodes | |
6. Low or absent natural killer cell activity | |
7. Ferritin ≥500 μg/L | |
8. Soluble CD25 (ie, soluble interleukin-2 receptor) ≥ 2400 U/ml |