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Objectives <p>To compare the diagnostic utility of the H-Score and modified HLH-2004 criteria in patients with suspected secondary HLH.</p> Materials and methods <p>A retrospective review of 10 secondary HLH patients diagnosed between August 2022 and July 2023 was conducted. Clinical, laboratory, and bone marrow findings were analyzed. Patients were classified by both modified HLH-2004 criteria (modified due to limited availability of NK cell function and sCD25) and the H-Score.</p> Results <p>Fever and hyperferritinemia were present in all patients. Cytopenias, hepatosplenomegaly, and marrow hemophagocytosis were frequent. Infections, particularly tuberculosis, hematological malignancies, and metabolic disorders were some of the triggers. Concordance between HLH-2004 criteria and H- Score was 70%. Importantly, the H-Score identified HLH in cases meeting only four HLH-2004 parameters. An H-Score cutoff ≥ 169 showed optimal sensitivity and specificity across both pediatric and adult subgroups.</p> Conclusion <p>The H-score identified a greater number of cases; however, whether these represent true cases requires further validation in larger studies. The absence of primary HLH cases, the inability to perform all investigations as per the HLH-2004 criteria, and the small sample size limit the generalizability of the study findings.</p>

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Optimizing Diagnosis of secondary HLH: Comparing H-Score and HLH-2004 Criteria

  • 期刊论文

  • 发表日期：

    2026年4月27日

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  • Komal Kela,
  • Kaninika Sanyal,
  • Mrinalini Kotru

摘要

Objectives

To compare the diagnostic utility of the H-Score and modified HLH-2004 criteria in patients with suspected secondary HLH.

Materials and methods

A retrospective review of 10 secondary HLH patients diagnosed between August 2022 and July 2023 was conducted. Clinical, laboratory, and bone marrow findings were analyzed. Patients were classified by both modified HLH-2004 criteria (modified due to limited availability of NK cell function and sCD25) and the H-Score.

Results

Fever and hyperferritinemia were present in all patients. Cytopenias, hepatosplenomegaly, and marrow hemophagocytosis were frequent. Infections, particularly tuberculosis, hematological malignancies, and metabolic disorders were some of the triggers. Concordance between HLH-2004 criteria and H- Score was 70%. Importantly, the H-Score identified HLH in cases meeting only four HLH-2004 parameters. An H-Score cutoff ≥ 169 showed optimal sensitivity and specificity across both pediatric and adult subgroups.

Conclusion

The H-score identified a greater number of cases; however, whether these represent true cases requires further validation in larger studies. The absence of primary HLH cases, the inability to perform all investigations as per the HLH-2004 criteria, and the small sample size limit the generalizability of the study findings.