Table 1.

Diagnostic criteria for the HLH-2004 trial, evaluated in this study

  • The diagnosis FHL can be established if at least 1 of either 1 or 2 below is fulfilled

  • A molecular diagnosis consistent with HLH

  • Diagnostic criteria for HLH fulfilled (5 of the 8 criteria below):

    • fever

    • splenomegaly

    • cytopenias (affecting ≥2/3 lineages in the peripheral blood:

    • hemoglobin <90 g/L; platelets <100 × 109/L; neutrophils <1.0 × 109/L [in infants aged <4 wk: hemoglobin <100 g/L])

    • hypertriglyceridemia and/or hypofibrinogenemia: fasting triglycerides ≥3.0 mmol/L; fibrinogen ≤1.5 g/L

    • hemophagocytosis

    • low or absent NK-cell activity (according to local laboratory reference)

    • ferritin ≥500 μg/L

    • sCD25 (ie, soluble interleukin-2 receptor) ≥2400 U/mL

 

  • The diagnosis FHL can be established if at least 1 of either 1 or 2 below is fulfilled

  • A molecular diagnosis consistent with HLH

  • Diagnostic criteria for HLH fulfilled (5 of the 8 criteria below):

    • fever

    • splenomegaly

    • cytopenias (affecting ≥2/3 lineages in the peripheral blood:

    • hemoglobin <90 g/L; platelets <100 × 109/L; neutrophils <1.0 × 109/L [in infants aged <4 wk: hemoglobin <100 g/L])

    • hypertriglyceridemia and/or hypofibrinogenemia: fasting triglycerides ≥3.0 mmol/L; fibrinogen ≤1.5 g/L

    • hemophagocytosis

    • low or absent NK-cell activity (according to local laboratory reference)

    • ferritin ≥500 μg/L

    • sCD25 (ie, soluble interleukin-2 receptor) ≥2400 U/mL

 
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