Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Apr 15;13(4):2388-2398.
eCollection 2021.

Analysis of cytokine risk factors in the early death of patients with secondary phagocytic lymphocytic histiocytosis

Zengzheng Li  1   2   3 , Jianqiong Liu  1   2   3 , Yan Man  1   2   3   4 , Fusheng Liu  1   2   3   4 , Lili Gao  1   2   3 , Peng Hu  1   2   3 , Renbin Zhao  1   2   3 , Yajie Wang  1   2   3   4 , Tonghua Yang  1   2   3   4
Affiliations

Analysis of cytokine risk factors in the early death of patients with secondary phagocytic lymphocytic histiocytosis

Zengzheng Li et al. Am J Transl Res. .

Abstract

Secondary hemophagocytic lymphohistiocytosis (sHLH) is an excessive inflammatory response syndrome caused by immune abnormalities. Up to date, the risk factors for cytokines causing early death in sHLH patients have not been elucidated. Our study reviewed the cytokine expression levels in peripheral blood of 50 sHLH patients. Through Cox proportional hazard model analysis, we found that IL-17F ≥2.835 pg/mL (HR = 5.922, 95% CI = 1.793-19.558, P = 0.004) was an independent death risk factor in sHLH patients, and it was also 30 days (Cutoff-value = 2.890 pg/mL, HR = 16.568, 95% CI = 1.917-143.195, P = 0.011), 60 days (Cutoff-value = 2.890 pg/mL, HR = 7.559, 95% CI = 1.449-39.423, P = 0.016), 90 day death risk factor (Cutoff-value = 2.835 pg/mL, HR = 7.649, 95% CI = 1.965-29.778, P = 0.003); IL-10 ≥16.730 pg/mL (HR = 4.821, 95% CI = 1.151-20.116, P = 0.031) is not only a death risk factor within 90 days, but also within 10 days (Cutoff-value = 944.350 pg/mL, HR = 13.321, 95% CI = 1.123-158.03, P = 0.027); and IL-5 ≥2.495 pg/mL (HR = 15.687, 95% CI = 1.377-178.645, P = 0.04) was also a death risk factor within 10 days. Besides, IL-17F, IL-10, IL-5, and the previously reported common risk factors Age, platelets, activated partial thromboplastin time, triglyceride, and lactate dehydrogenase were analyzed together. It was found that the patient age ≥56 years-old is was an important risk factor for death within 30 days, IL-17 ≥2.89 pg/mL and IL-10 ≥16.73 pg/mL are important risk factors for patient death. In summary, our data indicate that age, IL-10 and IL-17F are important risk factors for early death in sHLH patients.

Keywords: Secondary hemophagocytic lymphohistiocytosis; age; cytokine; interleukin 10; interleukin 17F; risk factors for early death.

PubMed Disclaimer

Conflict of interest statement

None.

Figures

Figure 1
Figure 1
Kaplan-Meier survival curve of HLH patients with Cytokine’s different risk between survivors and non-survivors. Kaplan-Meier curve of patients Overall survival (OS) with (A) IL-17A ≥1.93 pg/mL or IL-17A <1.93 pg/mL, (B) IL-1β <2.695 pg/mL or IL-1β ≥2.695 pg/mL, (C) IL-17F <2.835 pg/mL or IL-17F ≥2.835 pg/mL, (D) INF-γ <2.605 pg/mL or INF-γ ≥2.605 pg/mL, (E) TNF-α ≥1.265 pg/mL or TNF-α <1.265 pg/mL, (F) IL-17F <46.35 pg/mL or IL-17F ≥46.35 pg/mL.
See this image and copyright information in PMC

References

    1. Tavernier SJ, Athanasopoulos V, Verloo P, Behrens G, Staal J, Bogaert DJ, Naesens L, De Bruyne M, Van Gassen S, Parthoens E, Ellyard J, Cappello J, Morris LX, Van Gorp H, Van Isterdael G, Saeys Y, Lamkanfi M, Schelstraete P, Dehoorne J, Bordon V, Van Coster R, Lambrecht BN, Menten B, Beyaert R, Vinuesa CG, Heissmeyer V, Dullaers M, Haerynck F. A human immune dysregulation syndrome characterized by severe hyperinflammation with a homozygous nonsense Roquin-1 mutation. Nat Commun. 2019;10:4779. - PMC - PubMed
    1. zur Stadt U, Rohr J, Seifert W, Koch F, Grieve S, Pagel J, Strauss J, Kasper B, Nürnberg G, Becker C, Maul-Pavicic A, Beutel K, Janka G, Griffiths G, Ehl S, Hennies HC. Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in munc18-2 and impaired binding to syntaxin 11. Am J Hum Genet. 2009;85:482–492. - PMC - PubMed
    1. Mo W, Wei W, Sun Y, Yang Y, Guan Z, Li M, Zhu P, Chi Z. Application of blood and immunodeficiency gene detection in the diagnosis of hemophagocytic lymphohistiocytosis patients. Exp Hematol. 2019;78:62–69. - PubMed
    1. Luo ZB, Chen YY, Xu XJ, Zhao N, Tang YM. Prognostic factors of early death in children with hemophagocytic lymphohistiocytosis. Cytokine. 2017;97:80–85. - PubMed
    1. Zhao Y, Lu D, Ma S, Li L, Zhu J, Zhou D, Zheng Y, Yang X, Zhu L, Zhu M, Xie M, Sun J, Ye X, Xie W. Risk factors of early death in adult patients with secondary hemophagocytic lymphohistiocytosis: a single-institution study of 171 Chinese patients. Hematology. 2019;24:606–612. - PubMed

LinkOut - more resources