Table 3. Diagnostic criteria for... | American Society of Hematology

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

Familial disease/known genetic defect
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