The Prognostic Role of Plasma Fibrinogen in Adult Secondary Hemophagocytic Lymphohistiocytosis

Overview

Journal Orphanet J Rare Dis

Publisher Biomed Central

Specialty General Medicine

Date 2020 Nov 26

PMID 33239052

Citations 7

Authors

Guangli Yin

Changfeng Man

Jiayu Huang

Shengen Liao

Xin Gao

Tian Tian

Limin Duan

Ji Xu

Hongxia Qiu

Affiliations

Soon will be listed here.

Abstract

Background: In adult patients with secondary hemophagocytic lymphohistiocytosis (sHLH), no valid immune biomarker has been available for predicting the prognosis of untreated sHLH patients.

Methods: Circulating plasma levels of fibrinogen (FIB) were measured at diagnosis in 293 cases of adult sHLH. We categorized FIB levels into tertiles. Multivariable Cox proportional hazards models were used to evaluate the relationship between FIB and survival. Restricted cubic spline models and two-piecewise Cox proportional hazards models were used to address the nonlinear association between FIB and mortality.

Results: During a median follow-up of 52 (interquartile ranges, 18-221) days, 208 deaths occurred, with 137 deaths in malignancy-associated hemophagocytic lymphohistiocytosis (MHLH) and 71 deaths in non-malignancy-associated hemophagocytic lymphohistiocytosis (non-MHLH). After multivariable adjustment, compared with the highest tertile of FIB, the hazard ratios (HRs) with 95% confidence intervals (CIs) of survival for tertile 2 and tertile 1 were 1.06 (0.90-1.24) and 0.84 (0.71-0.98), respectively. The restricted cubic spline curve displayed a nonlinear and inverse relationship between FIB and mortality. Furthermore, the threshold effect analysis demonstrated that the inflection point for the curve was at an FIB level of 1.76 g/L. The HRs (95% CIs) for survival were 0.68 (0.55-0.83) and 1.08 (0.96-1.21) on the left and right side of the inflection point, respectively.

Conclusions: These results suggest that plasma fibrinogen is nonlinearly and inversely associated with the risk of mortality in adult secondary hemophagocytic lymphohistiocytosis.

Citing Articles

Development and validation of an early mortality risk model for pediatric hemophagocytic lymphohistiocytosis: a comparison with HScore, PELOD-2, P-MODS, and pSOFA.

Tang Z, Zhu D, Li X, Yan H, Luo T, Xie L Ann Hematol. 2024; 103(8):2699-2709.

PMID: 38736014 DOI: 10.1007/s00277-024-05780-2.

Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.

Nguyen T, Kim Y, Jeong G, Jin M Exp Mol Med. 2024; 56(3):559-569.

PMID: 38448692 PMC: 10984945. DOI: 10.1038/s12276-024-01182-6.

Association of a decreased platelet count with poor survival in patients with adult secondary hemophagocytic lymphohistiocytosis.

Cheng W, Xu J, Shu Y, Qiu H, Yin G Ann Hematol. 2024; 103(4):1159-1166.

PMID: 38378930 DOI: 10.1007/s00277-024-05663-6.

Hemophagocytic lymphohistiocytosis with jaundice as first manifestation: A case report.

Wang D, Wu S, Kong B, Song L World J Clin Cases. 2023; 11(34):8212-8218.

PMID: 38130789 PMC: 10731184. DOI: 10.12998/wjcc.v11.i34.8212.

A novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis.

Wang S, Lv K, Zhou Y, Cheng X, Chen Z, Shen H Ann Hematol. 2023; 102(11):3251-3259.

PMID: 37561154 PMC: 10567857. DOI: 10.1007/s00277-023-05398-w.

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