Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
Department of Cardiology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
Orphanet J Rare Dis. 2020 Nov 25;15(1):332. doi: 10.1186/s13023-020-01622-2.
In adult patients with secondary hemophagocytic lymphohistiocytosis (sHLH), no valid immune biomarker has been available for predicting the prognosis of untreated sHLH patients.
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.
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.
These results suggest that plasma fibrinogen is nonlinearly and inversely associated with the risk of mortality in adult secondary hemophagocytic lymphohistiocytosis.
在成人继发性噬血细胞性淋巴组织细胞增生症（sHLH）患者中，尚无有效的免疫生物标志物可用于预测未经治疗的 sHLH 患者的预后。
在 293 例成人 sHLH 患者的诊断时测量了循环血浆纤维蛋白原（FIB）水平。我们将 FIB 水平分为三分位。多变量 Cox 比例风险模型用于评估 FIB 与生存之间的关系。受限立方样条模型和两段式 Cox 比例风险模型用于解决 FIB 与死亡率之间的非线性关系。
在中位数为 52（四分位间距，18-221）天的随访期间，发生了 208 例死亡，其中恶性肿瘤相关噬血细胞性淋巴组织细胞增生症（MHLH）死亡 137 例，非恶性肿瘤相关噬血细胞性淋巴组织细胞增生症（非-MHLH）死亡 71 例。经过多变量调整后，与 FIB 最高三分位相比，FIB 第 2 三分位和第 1 三分位的生存风险比（HR）及其 95%置信区间（CI）分别为 1.06（0.90-1.24）和 0.84（0.71-0.98）。受限立方样条曲线显示了 FIB 与死亡率之间的非线性和反向关系。此外，拐点分析表明，曲线的拐点位于 FIB 水平为 1.76 g/L。拐点左侧和右侧的生存 HR（95%CI）分别为 0.68（0.55-0.83）和 1.08（0.96-1.21）。
这些结果表明，血浆纤维蛋白原与成人继发性噬血细胞性淋巴组织细胞增生症的死亡风险呈非线性反比关系。
