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. 2020 Jul 1;7(7):69. doi: 10.3390/children7070069

Table 2.

2004 diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH) [36].

HLH Diagnosis Can Be Established with either 1 or 2.

  1. Molecular diagnosis consistent with HLH

  2. Diagnostic criteria fulfilled by meeting five out of the eight criteria below
    1. Fever
    2. Splenomegaly
    3. Cytopenias affecting two out of three blood lineages in peripheral blood
      • Hemoglobin < 90 g/L (in infants < 4 weeks: hemoglobin < 100 g/L)
      • Platelets < 100 × 109/L
      • Neutrophils < 1.0 × 109/L
    4. Hypertriglyceridemia and/or hypofibrinogenemia:
      • Fasting triglycerides ≥ 3.0 mmol/L (i.e., ≥265 mg/dL)
      • Fibrinogen ≤ 1.5 g/L
    5. Hemophagocytosis in bone marrow or spleen or lymph nodes and no malignancy
    6. Low or absent NK cell activity (according to local laboratory reference)
    7. Ferritin ≥ 500 mg/L
    8. Soluble CD25 (i.e., soluble IL-2 receptor) > 2400 U/mL

Abbreviations: dL, deciliter; g, grams; IL, interleukin; L, liter; mg, milligrams; mL, milliliter; mmol, millimoles; U, units.