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Table 2 Diagnostic criteria for hemophagocytic syndrome according to trial HLH-2004

From: Acquired hemophagocytic syndrome related to parainfluenza virus infection: case report

Confirmed molecular disease: PRF1 mutation, UNC13D, Munc 18-2, Rab27a, STX11, SH2D1A, BIRC4
Five of eight clinical and laboratory criteria: 1. Fever
  2. Splenomegaly
  3. Cytopenia affecting two or more cell lines:
  • Hemoglobin <9g/dL
  • Platelets <100,000/mL
  • Neutrophils <1000/mL
  4. Hypertriglyceridemia (fasting triglycerides >265mg/dL or 3mMol/L) and or hypofibrinogenemia (<150mg/dL)
  5. Hemophagocytosis in bone marrow, spleen, liver, lymph node, skin
  6. Decreased or absent natural killer cell activity
  7. Ferritin >500ng/mLa
  8. Soluble CD25 >2400U/mL
  1. aFerritin greater than 10,000ng/mL is almost confirmatory.