Diagnostic criteria used in HLH-94
| Criteria |
| Clinical |
| Fever |
| Splenomegaly |
| Laboratory |
| Cytopenias (affecting at least 2 of 3 lineages in the peripheral blood) |
| Hemoglobin (< 90 g/L), platelets (< 100 × 109/L), neutrophils (< 1.0 × 109/L) |
| Hypertriglyceridemia and/or hypofibrinogenemia |
| Fasting triglycerides ≥ 2mM or ≥ 3 SDs of the normal value for age |
| Fibrinogen ≤ 1.5 g/L or ≤ 3 SDs of the normal value for age |
| Histopathologic |
| Hemophagocytosis in the BM or spleen or lymph node |
| No evidence of malignancy |
| Criteria |
| Clinical |
| Fever |
| Splenomegaly |
| Laboratory |
| Cytopenias (affecting at least 2 of 3 lineages in the peripheral blood) |
| Hemoglobin (< 90 g/L), platelets (< 100 × 109/L), neutrophils (< 1.0 × 109/L) |
| Hypertriglyceridemia and/or hypofibrinogenemia |
| Fasting triglycerides ≥ 2mM or ≥ 3 SDs of the normal value for age |
| Fibrinogen ≤ 1.5 g/L or ≤ 3 SDs of the normal value for age |
| Histopathologic |
| Hemophagocytosis in the BM or spleen or lymph node |
| No evidence of malignancy |