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Clinical calculator summary

HScore for HLH

The HScore is a validated tool to estimate the probability of secondary HLH in adults.

Evidence-based context for fast calculator use

Purpose:
HScore calculates the probability of hemophagocytic lymphohistiocytosis (HLH) diagnosis to guide initiation of immunosuppressive therapy.
Population:
patients with suspected or confirmed histiocytic or hyperinflammatory disease matching the original criteria
Factors:
Known Immunosuppression?, Maximal Temperature, Organomegaly, Number of Cytopenias, Ferritin, Triglycerides, Fibrinogen, SGOT / AST
Reference:
Fardet L, et al. Development and validation of the HScore, a score for the diagnosis of hemophagocytic lymphohistiocytosis. Arthritis Rheumatol. 2014;66(9):2613-2620.
HomeHScore for HLH
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HScore for HLH

Clinical Context & Background

The HScore is a validated tool to estimate the probability of secondary HLH in adults. It incorporates clinical, biologic, and cytologic features. A score > 169 correlates with a > 93% sensitivity and > 86% specificity for HLH.
Primary Source EvidenceFardet L, et al. Development and validation of the HScore, a score for the diagnosis of hemophagocytic lymphohistiocytosis. Arthritis Rheumatol. 2014;66(9):2613-2620.
Formula Logic
Sum of weighted points for 9 variables.

Reference Data

HScoreProbability of HLH
< 90< 1%
90 - 169Low to Intermediate
169~ 70% cutoff
> 200> 98%

Clinical Workflow

Use, Interpret, And Continue The Patient Pathway

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

When To Use

  • Use HScore for HLH when hScore calculates the probability of hemophagocytic lymphohistiocytosis (HLH) diagnosis to guide initiation of immunosuppressive therapy.
  • 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 < 90 through > 200.
  • 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 Known Immunosuppression?, Maximal Temperature, Organomegaly, 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 HScore for HLH 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 HScore for HLH determine treatment by itself?

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

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