Table 1

Diagnostic guidelines for HLH

Guidelines
The diagnosis of HLH requires a molecular diagnosis consistent with HLH or 5 of 8 of the below criteria 
 1. Fever 
 2. Splenomegaly 
 3. Cytopenias affecting ≥2 lineages 
  a. Hemoglobin <9 g/dL 
  b. Platelets <100 × 109/L 
  c. Neutrophils <1.0 × 109/L 
 4. Hypertriglyceridemia and/or hypofibrinogenemia 
  a. Triglycerides ≥265 mg/dL 
  b. Fibrinogen ≤150 mg/dL 
 5. Hemophagocytosis in bone marrow, spleen, or lymph nodes 
 6. Low or absent NK cell activity 
 7. Ferritin ≥500 µg/L 
 8. sCD25 (ie, sIL2R) ≥2400 U/mL 
Guidelines
The diagnosis of HLH requires a molecular diagnosis consistent with HLH or 5 of 8 of the below criteria 
 1. Fever 
 2. Splenomegaly 
 3. Cytopenias affecting ≥2 lineages 
  a. Hemoglobin <9 g/dL 
  b. Platelets <100 × 109/L 
  c. Neutrophils <1.0 × 109/L 
 4. Hypertriglyceridemia and/or hypofibrinogenemia 
  a. Triglycerides ≥265 mg/dL 
  b. Fibrinogen ≤150 mg/dL 
 5. Hemophagocytosis in bone marrow, spleen, or lymph nodes 
 6. Low or absent NK cell activity 
 7. Ferritin ≥500 µg/L 
 8. sCD25 (ie, sIL2R) ≥2400 U/mL 
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