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