Abstract
BackgroundIn adult patients with secondary hemophagocytic lymphohistiocytosis (sHLH), no valid immune biomarker has been available for predicting the prognosis of untreated sHLH patients.MethodsCirculating 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.ResultsDuring 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.ConclusionsThese results suggest that plasma fibrinogen is nonlinearly and inversely associated with the risk of mortality in adult secondary hemophagocytic lymphohistiocytosis.
Highlights
In adult patients with secondary hemophagocytic lymphohistiocytosis, no valid immune biomarker has been available for predicting the prognosis of untreated sHLH patients
Distribution of plasma fibrinogen and patient characteristics The baseline characteristics for patients stratified by FIB tertiles are listed in Table 1.The mean age of the total study population was 53 (41–64) years, and 62.5% were male
The participants with lower FIB had lower platelet levels and higher Alanine transaminase (ALT), Aspartate transaminase (AST), Lactate dehydrogenase (LDH), TG, β2-MG, serum ferritin, and Soluble interleukin-2 receptor (sCD25) levels and total Hscore points and were more likely to be infected with Epstein-Barr virus (EBV)
Summary
In adult patients with secondary hemophagocytic lymphohistiocytosis (sHLH), no valid immune biomarker has been available for predicting the prognosis of untreated sHLH patients. One pediatric HLH study reported that plasma FIB levels (< 150 mg/dL) at diagnosis may be prognostic measures of inferior survival [8]. Several retrospective studies have reported the prognostic role of plasma fibrinogen in adult sHLH [4, 9]. These studies were limited by a relatively small sample size (< 120 patients) and lacked follow-up data or adjusted confounding factors influencing prognosis for sHLH patients [9, 10].
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