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Prognostic factors in patients with secondary hemophagocytic lymphohistioc ytosis in a Chinese cohort

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome with high mortality mediated by an unbridled and persistent activation of cytotoxic T lymphocytes and natural killer cells. However, the influence factors of early death in adult sHLH patients are still not fully elucidated, which need further investigating. We have conducted an observational study of adult HLH patients between January 2016 and December 2022. All patients are enrolled according to HLH-2004 criteria. Clinical manifestations, laboratory data, treatments, and outcomes have been recorded. Influence factors associated with prognosis are calculated by using logistic regression models. Overall, 220 patients enrolled in this study. The etiologies of HLH were divided into five groups including autoimmune-associated hemophagocytic syndrome (AAHS) (n = 90, 40.9%), malignancies (n = 73, 33.2%), EBV-HLH (n = 18, 8.2%), infection excluded EBV (n = 24, 10.9%), and other triggers (n = 15, 6.8%). Among them, EBV-HLH had the highest mortality (77.8%), and AAHS had the lowest mortality (14.4%). Multivariate analysis indicated that age (≥ 38 years old), cytopenia ≥ 2 lines, platelets (≤ 50 × 109/L), aspartate aminotransferase (≥ 135U/L), prothrombin time (≥ 14.9 s) and activated partial thromboplastin time (≥ 38.5s), EBV, and fungal infection are independent risk factors for poor prognosis of HLH. Adult HLH patients with elder age, cytopenia ≥ 2 lines, levels of decreased platelets, increased AST, prolonged PT and APTT, EBV, and fungal infection tend to have a poor prognosis.

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The datasets used or analyzed about the study could be available from the corresponding author on reasonable request.

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Funding

The study was supported by Beijing Natural Science Foundation, under project no. 7234393.

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Authors and Affiliations

  1. Department of Emergency, Peking University People’s Hospital, Beijing, China

    Yuanyuan Pei, Jihong Zhu & Lingjie Cao

  2. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China

    Ranran Yao, Ziye Wang, Renge Liang, Yuan Jia & Yin Su

Authors
  1. Yuanyuan Pei
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  2. Jihong Zhu
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  3. Ranran Yao
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  4. Lingjie Cao
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  5. Ziye Wang
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  6. Renge Liang
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  7. Yuan Jia
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  8. Yin Su
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Contributions

Contribution to the concept or design of the work was carried out by Yuanyuan Pei, Jihong Zhu, Yuan Jia, and Yin Su. Access to informed consent was carried out by Yuanyuan Pei, Ranran Yao, Lingjie Cao, Renge Liang, and Ziye Wang. Yuanyuan Pei, Ranran Yao, and Renge Liang participated in clinical data entry and statistics. Yuanyuan Pei was responsible for data statistical analysis and article writing. Jia Yuan and Yin Su were responsible for the revision of this article. All authors read and approved the final manuscript.

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Correspondence to Yuan Jia or Yin Su.

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Pei, Y., Zhu, J., Yao, R. et al. Prognostic factors in patients with secondary hemophagocytic lymphohistioc ytosis in a Chinese cohort. Ann Hematol 103, 695–703 (2024). https://doi.org/10.1007/s00277-023-05567-x

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  • DOI: https://doi.org/10.1007/s00277-023-05567-x

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