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Clinical calculator summary

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.

Evidence-based context for fast calculator use

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.
Primary Source EvidenceHenter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124-131.
Formula Logic
Diagnosis requires ≥ 5 of 8 criteria.

Reference Data

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

Clinical Workflow

Use, Interpret, And Continue The Patient Pathway

Expand for workflow guidance, limitations, examples, and related next steps.

When To Use

  • Use HLH-2004 Diagnostic Criteria Calculator — Hemophagocytic Lymphohistiocytosis when apply the HLH-2004 diagnostic criteria (5 of 8 criteria) for hemophagocytic lymphohistiocytosis. Systematic evaluation tool based on the Histiocyte Society diagnostic guidelines.
  • Confirm that the patient, diagnosis, disease phase, and available inputs match the cited model before calculation.

How To Interpret

  • Interpret the displayed result using the calculator-specific formula and reference table, spanning 0 - 4 through 5 - 8.
  • A boundary result should prompt input verification and clinical review rather than false precision.

What To Do Next

  • Confirm pathology, organ involvement, infectious and malignant mimics, laboratory trends, and urgent specialist input.
  • Document the inputs, result, timing, and clinical context so the assessment can be reproduced.

Limitations

  • Classification and hyperinflammation scores do not replace diagnostic criteria or emergency treatment assessment.
  • The result supports clinician judgment and does not independently determine treatment.

Validated Population

patients with suspected or confirmed histiocytic or hyperinflammatory disease matching the original criteria

How to apply this result

For a representative case, verify Fever, Splenomegaly, Cytopenias affecting ≥ 2 lineages, calculate the result, and confirm that its classification matches the highlighted reference band before continuing the disease-specific pathway.

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Frequently Asked Questions

When should HLH-2004 Diagnostic Criteria Calculator — Hemophagocytic Lymphohistiocytosis be used?

Use it for patients with suspected or confirmed histiocytic or hyperinflammatory disease matching the original criteria when all required inputs and the intended clinical setting are confirmed.

Can HLH-2004 Diagnostic Criteria Calculator — Hemophagocytic Lymphohistiocytosis determine treatment by itself?

No. Interpret the result with the cited evidence, complete clinical assessment, current guidelines, and patient-specific goals.

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