Table 1

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