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Prognostic Value of Blood-Based Inflammatory Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis

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Abstract

Purpose

Hemophagocytic lymphohistiocytosis (HLH) is a rare systematic immune disease manifested with excessive activation of lymphocytes and macrophages. This study was designed to explore the feasible prognostic factors of secondary HLH (sHLH).

Method

We retrospectively analyzed 179 patients with newly diagnosed sHLH from January 2016 to May 2019 according to the HLH-2004 protocol. Baseline characteristics and laboratory results were reviewed.

Results

The median age of all patients was 53 years, with a male/female ratio of 1.45. The commonest cause of HLH was malignancy. Of the 179 patients, 48.6% presented with Epstein-Barr virus (EBV) infection, 92.8% with hemocytopenia (at least 2 lineages), 60.3% with hypofibrinogenemia, 43.0% with hypertriglyceridemia (≥ 3 mmol/L), 99.4% with high ferritin, 97.8% with fever, 72.1% with splenomegaly, and 72.6% with hemophagocytosis. As to their prognosis, 122 patients died; the median survival was 88 days, with a 2-year survival rate of 26.72%. Univariate analysis confirmed neutrophil-to-lymphocyte ratio ˃ 2.53, lymphocyte-to-monocyte ratio (LMR) ≤ 4.43, platelet-to-lymphocyte ratio ˃ 227.27, red blood cell distribution width ˃ 14.6, red blood cell distribution width-to-platelet ratio (RPR) > 0.33, EBV infection, platelet ≤ 34 × 109 /L, fibrinogen ≤ 1.34 g/L, alkaline phosphatase ˃ 182.4 U/L, adenosine deaminase ˃ 69.2 U/L, and ferritin ˃ 2318 ng/mL were associated with an inferior survival. In a multivariate model, LMR, RPR, and ferritin were considered as three independent factors.

Conclusion

Some blood-based inflammatory markers, which can be easily and cheaply detected, are significantly associated with the OS of HLH patients. LMR and RPR, superior to NLR, PLR, RDW, can be taken to predict the OS of patients with HLH.

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Funding

This work was supported by the National Natural Science Foundation of the People’s Republic of China [No. 81570175].

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Authors and Affiliations

  1. Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China

    Jiayu Huang, Guangli Yin, Wanying Cheng, Lingling Liu & Hongxia Qiu

  2. Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China

    Limin Duan, Tian Tian, Ji Xu, Jujuan Wang, Xin Gao & Hongxia Qiu

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  1. Jiayu Huang
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  2. Guangli Yin
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  3. Limin Duan
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  4. Tian Tian
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  5. Ji Xu
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Contributions

This study was conceived and designed by JH and HQ. JH, GY, WC, and LL contributed to collect retrospective research data, as well as JH, LD, TT, and JX support to analyze or interpret these data. JH draft the manuscript and GY, JW, XG, and HQ helped revise the manuscript strictly for important content. All authors have read and approved the final manuscript.

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Correspondence to Hongxia Qiu.

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All procedures in studies were performed in accordance with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.

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Huang, J., Yin, G., Duan, L. et al. Prognostic Value of Blood-Based Inflammatory Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis. J Clin Immunol 40, 718–728 (2020). https://doi.org/10.1007/s10875-020-00801-x

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