Table 3.

Diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH).*

Supportive evidence are cerebral symptoms with moderate pleocytosis and/or elevated protein, elevated transaminases and bilirubin, LDH > 1000 U/L 
*Janka and Schneider1  
§ for methods see38  
Reprinted with permission from Blackwell Publishing, Oxford 

  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 ≥ 2400 U/mL §

    • Decreased or absent NK-cell activity

    • Hemophagocytosis in bone marrow, CSF or lymph nodes

 
Supportive evidence are cerebral symptoms with moderate pleocytosis and/or elevated protein, elevated transaminases and bilirubin, LDH > 1000 U/L 
*Janka and Schneider1  
§ for methods see38  
Reprinted with permission from Blackwell Publishing, Oxford 

  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 ≥ 2400 U/mL §

    • Decreased or absent NK-cell activity

    • Hemophagocytosis in bone marrow, CSF or lymph nodes

 
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