Table 2. Diagnostic criteria of HLH-2004 trial.
[8]
HLH: hemophagocytic lymphohistiocytosis
| Molecular identification of an HLH-associated gene mutation: | Or | 5 of 8 of the following criteria: |
| Children require documentation of homozygosity or compound heterozygosity for HLH-associated gene mutations | 1. Fever | |
| 2. Splenomegaly | ||
| 3. Cytopenia affecting 2 lines | ||
| Adults: heterozygosity may be sufficient if they have clinical findings associated with HLH | a. Hemoglobin <9 g/dl | |
| b. Platelets <100 k/μl | ||
| c. Neutrophils <1.0 x109/l | ||
| 4.Hypertriglyceridemia and/or hypofibrinogenemia | ||
| a. Triglycerides > 265 mg/dl | ||
| b. Fibrinogen<150 mg/dl | ||
| 5. Hemophagocytosis in bone marrow, spleen, liver or lymph nodes | ||
| 6. Low or absent natural killer cell activity | ||
| 7. Ferritin > 500 ng/ml | ||
| 8. CD25 >2400 U/mL |