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HLH-2004 Diagnostic Criteria

The HLH-2004 criteria define the diagnostic requirements for hemophagocytic lymphohistiocytosis, requiring 5 of 8 clinical and laboratory criteria to establish the diagnosis.

Purpose:
Diagnose hemophagocytic lymphohistiocytosis (HLH) using standardised criteria
Population:
Patients with suspected HLH (children and adults)
Factors:
Fever, Splenomegaly, Cytopenias (2-3 lineages), Hypertriglyceridaemia/hypofibrinogenaemia, Haemophagocytosis, Low/absent NK cell activity, Ferritin ≥500 μg/L, Elevated soluble CD25
Reference:
Henter et al., Pediatric Blood & Cancer 2007
HomeHLH-2004 Diagnostic Criteria Calculator — Hemophagocytic Lymphohistiocytosis
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HLH-2004 Diagnostic Criteria Calculator — Hemophagocytic Lymphohistiocytosis

Clinical Context & Background

The HLH-2004 guidelines establish the clinical diagnosis of Hemophagocytic Lymphohistiocytosis (HLH). A molecular diagnosis is consistent with HLH. In the absence of a known genetic mutation, a diagnosis can be made if at least 5 of the 8 clinical and laboratory criteria are met.
Formula Logic
Diagnosis requires ≥ 5 of 8 criteria.

Reference Data

Criteria MetInterpretation
0 - 4Not Diagnostic (unless genetic proof exists)
5 - 8Diagnostic for HLH

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Evidence-based oncology decision support. Verify with clinical guidelines.