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Clinical analysis and a novel risk predictive nomogram for 155 adult patients with hemophagocytic lymphohistiocytosis

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Abstract

Recently, more and more attention has been paid on adult hemophagocytic lymphohistiocytosis (HLH), a disease with complicated symptoms and high mortality. In order to analyze the clinical characteristics and prediction risk factors of mortality, we designed a retrospective study with 1-year follow-up and included 155 patients admitted to Tongji Hospital diagnosed as HLH. One hundred seven patients formed the training cohort for nomogram development, and 48 patients formed the validation cohort to confirm the model’s performance. All patients’ clinical characteristics, laboratory results, medical records, and prognosis were analyzed. Among all the 107 patients in the training cohort, 46 were male and 61 were female, with the median age of 49.0 (IQR 31.0–63.0). The 1-year mortality rate was 43.9% (47/107) and 45.8% (22/48) in the training and validation cohort, respectively. And further multivariate logistic regression analysis in the training cohort showed that male (odds ratio 5.534, 95% CI 1.507–20.318, p = 0.010), altered mental status (11.876, 1.882–74.947, p = 0.008), serum ferritin ≥ 31,381 μg/L (8.273, 1.855–36.883, p = 0.006), and IL-6 ≥ 18.59 pg/mL (19.446, 1.527–247.642, p = 0.022) were independent risk factor of mortality. A nomogram included the four prediction factors mentioned above was also tabled to help clinicians evaluate the probability of poor outcome. Area under the receiver operating characteristic curve (AUROC) analysis, calibration curves, and decision curve analysis (DCA) certify the accuracy and the clinical usefulness of the nomogram. Our research reveals that male, altered mental status, serum ferritin ≥ 31,381 µg/L, and IL-6 ≥ 18.59 pg/mL are four independent predictors for poor prognosis. Doctors should pay more attention to patients with altered mental status, high serum ferritin, and IL-6 level, who have a higher risk of death.

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Abbreviations

HLH:

Hemophagocytic lymphohistiocytosis

HPS:

Hemophagocytic syndrome

MAS:

Macrophage activation symptoms

ALT:

Alanine aminotransferase

AST:

Aspartate aminotransferase

TBil:

Total bilirubin

ALB:

Albumin

PT:

Prothrombin time

TT:

Thrombin time

sIL-2R:

Soluble IL-2 receptor

IL-6:

Interleukin-6

hsCRP:

High sensitive C-reactive protein

PCT:

Procalcitonin

SF:

Serum ferritin

LDH:

Lactate dehydrogenase

ESR:

Erythrocyte sedimentation rate

eGFR:

Estimated glomerular filtration rate

AMS:

Altered mental status

IQR:

Interquartile range

OR:

Odds ratio

CI:

Confidence interval

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Acknowledgements

Authors expressed their gratitude for all persons who were involved in reporting patients and gathering data.

Funding

This work is funded by the Chinese National Thirteenth Five Years Project in Science and Technology (2017ZX10202201).

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Authors and Affiliations

  1. Department and Institute of Infectious Disease, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China

    Mengxin Lu, Yanghao Xie, Xiaoxu Guan, Ming Wang, Lin Zhu, Shen Zhang, Qin Ning & Meifang Han

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  1. Mengxin Lu
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  2. Yanghao Xie
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Contributions

M-X L and X-X G designed the study. All the authors contributed to the generation, collection, assembly, analysis of the data. M-X L and Y-H X wrote the manuscript; M-F H and Q N revised the manuscript. All the authors have read manuscript and approved the final manuscript.

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Correspondence to Qin Ning or Meifang Han.

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Ethics approval

This retrospective chart review study was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Ethical Committee of Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, Wuhan, China approved this study, as retrospective design Ethical Committee abandoned the need for informed consent.

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The authors declare no competing interests.

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Mengxin Lu and Yanghao Xie are co-first authors; they contributed equally to the work.

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Lu, M., Xie, Y., Guan, X. et al. Clinical analysis and a novel risk predictive nomogram for 155 adult patients with hemophagocytic lymphohistiocytosis. Ann Hematol 100, 2181–2193 (2021). https://doi.org/10.1007/s00277-021-04551-7

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