- HLH-2004 criteria
- HScore
| Parameter | HLH-2004 diagnostic criteria | HScore | Comments |
|---|---|---|---|
| Known underlying immunosuppression | NA | 0 (no) or 18 (yes) | HIV positive or receiving long‐term immunosuppressive therapy (e.g., glucocorticoids, cyclosporine). |
| Fever | 0 (<38.5) or 1 (38.5) | 0 (39.4) | Persistent or recurrent high fever. Nearly universal in untreated HLH. |
| Hepatomegaly | NA | 0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly | Abnormal liver tests reported in about 80% of adults with HLH (hypoalbuminemia detected in most adults with HLH). |
| Splenomegaly | 0 (no) or 1 (yes) | 0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly) | Splenomegaly may be caused by infiltration by activated lymphocytes and histiocytes or it by an underlying condition triggering HLH. |
| Cytopenias | 0 (one lineage) or 1 (two or three lineages)* | 0 (one lineage), 24 (two lineages), or 34 (three lineages) | Especially thrombocytopenia and anemia in early stages; results primarily from severe cytokine-mediated myelosuppression. |
| High Ferritin level (ng/ml) | 0 (<500) or 1 (500) | 0 (6,000) | No specific ferritin level determined to be specific for HLH. However, the higher the ferritin the likelier the diagnosis of HLH. |
| Elevated triglyceride level | NA | 0 (4) | Hypertriglyceridemia is detected in 42%-85% of adults. |
| Low Fibrinogen level | NA | 0 (>2.5) or 30 (<2.5) | Hypofibrinogenemia detected in about 50% of adults with HLH. |
| Elevated triglyceride level or low fibrinogen level | Triglycerides > 265 mg/dl and/or fibrinogen </= 15 mg/dL (1) | NA | |
| AST | NA | 0 (<30) or 19 (30) | LDH and bilirubin may also be increased. |
| Hemophagocytosis features on bone marrow aspirate | 0 (no) or 1 (yes) | Hemophagocytosis in bone marrow 0 (no) or 1 (yes) | If the bone marrow specimen is not conclusive, material may be obtained from other organs. |
| Elevated soluble IL-2 receptor | 1 (sIL-2R ≥ 2,400 units/mL) | NA | sIL-2R (sCD25), a marker of T cell activation; not available at most medical centers, their utility in real-time diagnosis and monitoring remains limited. |
| Low or absent NK-cell activity | 1 (Yes) | NA | Not available at most medical centers; their utility in real-time diagnosis and monitoring remains limited. |
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