Diagnostic criteria of hemophagocytic lymphohistiocytosis: HLH-2004.
| Diagnosis will be established if one of either (1) or (2) is fulfilled |
|---|
| (1) Molecular diagnosis consistent with HLH |
| (2) Diagnostic criteria for HLH fulfilled (5 out of the 8 criteria shown below) |
| ① Fever ≥38.5°C for ≥7 days |
| ② Splenomegaly ≥3 finger breadth below the left subcostal margin |
| ③ Cytopenias affecting ≥2 of 3 lineages in peripheral blood |
| Hemoglobin <9 g/L |
| Platelets <100×109/L |
| Absolute neutrophil count <1.0×109/L |
| ④ Hypertriglyceridemia and/or hypofibrinogenemia |
| Fasting triglycerides ≥265 mg/dL, Fibrinogen ≤1.5 g/L |
| ⑤ Hemophagocytosis in the bone marrow or spleen or lymph node |
| ⑥ Low or absent NK cell activity (according to the local laboratory reference) |
| ⑦ Ferritin ≥500 μg/L |
| ⑧ Soluble CD25 (sIL-2 receptor) ≥2,400 U/mL |
Abbreviations: HLH, hemophagocytic lymphohistiocytosis; NK, natural killer; sIL-2, soluble interleukin-2.
Blood Res 2021;56:S17~S25 https://doi.org/10.5045/br.2021.2020323
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