Table 1 : Diagnostic criteria for hemophagocytic lymphohistiocytosis.

  1. Familial disease/known genetic defect
  2. Clinical and laboratory criteria (5/8 criteria)
    • Fever
    • Splenomegaly
    • Cytopenia ≥2 cell lines
    • Hemoglobin <90 g/L (below 4 weeks <120 g/L)
    • Neutrophils <1×109/L
    • Hypertriglyceridemia and/or hypofibrinogenemia
    • Fasting triglycerides ≥3 mmol/L
    • Fibrinogen <1.5 g/L
    • Ferritin ≥500 μg/L
    • sCD25 ≥2,400 U/mL
    • Decreased or absent NK cell activity
    • Hemophagocytosis in bone marrow, CSF, or lymph nodes
Vrotsos et al.Journal of Cancer Therapeutics and Research  2014 3:7DOI : 10.7243/2049-7962-3-7