Table 1.
HLH-2004 diagnostic criteria and patient-specific criteria identified.
| HLH criteria: | Patient #1 | Patient #2 | Patient #3 | Patient #4 |
|---|---|---|---|---|
| 1. Fever: ≥38.5°C | Yes | Yes | Yes | Yes |
| 2. Splenomegaly | Yes | Yes | Yes | Yes |
| 3. Cytopenia affecting ≥ 2 lineages: - Anemia: Hb <9 g/dl - Thrombocytopenia: platelets <100 k/µl - Neutropenia: ANC <1,000 |
Yes ✓ ✓ ✓ |
Yes ✓ ✓ |
Yes ✓ ✓ ✓ |
Yes ✓ ✓ |
| 4. Hypertriglyceridemia: fasting triglycerides >265 mg/dl
|
NA Yes |
No Yes |
Yes Yes |
No No |
| 5. Hemophagocytosis in bone marrow, spleen, or lymph nodes |
Yes | NA | Yes | No |
| 6. Hyperferrtinemia: >500 µg/L | NA | Yes | Yes | Yes |
| 7. Low or absent NK-cell activity | NA | NA* | NA* | NA |
| 8. Elevated soluble IL-2R/CD25: ≥2,400 U/ml |
NA | Yes | Yes | Yes |
✓cytopenia identified; NA, not available/not attained; *lab drawn, but insufficient sample.