Table 3.
Proposed criteria for diagnosing macrophage activation syndrome
| HLH-2004 criteria32 | Ravelli et al.24 | Kostik et al.26 |
|---|---|---|
| Either criterion 1 or 2 fulfilled | ≥2 laboratory criteria or ≥2 clinical and/or laboratory criteria | ≥3 laboratory criteria |
| 1. Molecular diagnosis consistent with primary HLH | Laboratory criteria: | • Low platelet count (≤211 × 109/L) |
| 2. At least 5 of 8 diagnostic criteria fulfilled | • Low platelet count (<262 × 109/L) | • Low WBC count (≤9.9 × 109/L) |
| • Persistent fever (>38.5°C) for at least 7 days | • Elevated AST (>59 U/L) | • Elevated AST (>59.7 U/L) |
| • Splenomegaly | • Low WBC count (≤4.0 × 109/L) | • Elevated LDH (>882 U/L) |
| • Cytopenias of ≥2 of 3 cell lines (hemoglobin <9.0 g/dL, platelets <100,000/μL, neutrophils <1,000/μL) | • Low fibrinogen (≤2.5 g/L) | • Low albumin (≤2.9 g/dL) |
| • Hypertrigyceridemia (fasting triglycerides ≥160mg/dL) or low fibrinogen (≤150 mg/dL) | Clinical criteria: | • Elevated ferritin (>400 μg/L) |
| • Serum ferritin ≥500 μg/L | • Central nervous system dysfunction (i.e., headache, seizures, coma, irritability, lethargy) | • Low fibrinogen (≤1.8 g/L) |
| • Low or absent NK cell activity | • Hemorrhages (mucosal bleeding, bruising, purpura) | • Proteinuria |
| • Elevated serum sIL-2R >2,400 IU/mL | • Hepatomegaly (≥3 cm below costal margin) | |
| • Hemophagocytosis in bone marrow, spleen, or lymph nodes |
Note
AST: aspartate aminotransferase; HLH: hemophagocytic lymphohistiocytosis; LDH: lactate dehydrogenase; NK: natural killer; sIL-2R: soluble interleukin 2 receptor; WBC: white blood cell.