Objective: To explore the clinical correlation and prognostic value of the Albumin-Bilirubin (ALBI) score in children with secondary hemophagocytic syndrome(sHLH).
Methods: A retrospective analysis was conducted on the data of children's sHLH cases clearly diagnosed in the Affiliated Hospital of Zunyi Medical University from January 2012 to March 2023. Survival analysis was conducted according to the ALBI classification. Spearman correlation analysis was conducted between the ALBI score and clinical indicators. The Receiver Operating Characteristic(ROC) curve was used to evaluate the ALBI score, select the best cutoff value, and evaluate the accuracy of prognostic prediction value. Kaplan-Meier method was used to draw the survival curve. Log-rank method was used to compare the differences of survival curve between groups. Cox regression was used for prognostic analysis and restricted cubic spline curves used to calculate the relationship between ALBI scores and the risk of death in children with sHLH.
Results: A total of 128 children with sHLH were included in this study, with a median age of 38(13.25, 84) months. There were 70 males (54.69%) and 58 females (45.31%). The survival analysis results of ALBI grading showed that the survival rate of HLH patients with ALBI grade 3 was significantly lower than those with ALBI grades 1 and 2. Spearman correlation analysis results showed that ALBI score was positively correlated with splenomegaly, respiratory failure, disseminated intravascular coagulation(DIC), pulmonary hemorrhage, gastrointestinal hemorrhage, central nervous system involvement, ALT, AST, TG, LDH, PT, APTT, and SF (the correlation coefficients are: r =0.181, 0.362, 0.332, 0.221, 0.351, 0.347, 0.391, 0.563, 0.180, 0.448, 0.483, 0.37, 0.356), and was negatively correlated with HB, PLT, and FIB (the correlation coefficients are: r =-0.321, -0.316, -0.423), but was not significantly correlated with EBV infection, fungal infection, hepatomegaly, and ANC (P ＞0.05). Using the ROC curve, the cutoff value of ALBI was -1.76. Single factor Cox regression analysis results showed that HB< 90 g/L, ALT≥80 U/L, AST≥200 U/L, LDH≥1 000 U/L, PT≥20 s, APTT≥40 s, FIB< 1.5 g/L, ALBI≥-1.76, combined pulmonary hemorrhage, DIC, central nervous system involvement, gastrointestinal bleeding, and not using blood purification may be the prognostic risk factors for children with sHLH (P < 0.05). Multivariate Cox regression results showed that FIB< 1.5 g/L (HR =2.119, 95% CI :1.028-4.368), ALBI≥-1.76 (HR =2.452, 95%CI :1.233-4.875), and central nervous system involvement (HR=4.674, 95%CI :2.486-8.789) were independent risk factors affecting prognosis, while blood purification (HR =0.306, 95%CI :0.153-0.612) was an independent protective factor for prognosis. The application of restricted cubic splines shows that the risk of death increases with the increase of ALBI score. The area under the ROC curve (AUC) of the ALBI score for predicting the risk of 1-week, 2-week, 4-week, and overall mortality were 0.825, 0.807, 0.700, and 0.693, respectively, indicating good predictive performance for early mortality risk. According to subgroup analysis results of clinical manifestations, compared with the ALBI < -1.76 group, ALBI≥-1.76 was associated with age ≤2 years, EBV infection, HLH-1994/2004 treatment, concomitant respiratory failure, and ANC≤1.0×10 9/L, HB< 90 g/L, PLT < 100×109/L, TG≥3.0 mmol/L, LDH≥1 000 U/L, APTT≥40 s, and FIB< 1.5 g/L (P < 0.05).
Conclusion: The ALBI score is related to the clinical characteristics and laboratory indicators of sHLH, and can be used as a beneficial indicator for assessing the prognostic risk of sHLH in children. It has good accuracy and clinical application value in predicting the prognosis of sHLH in children.
题目: ALBI评分在儿童继发性噬血细胞综合征中的临床相关性及预后分析.
目的: 探讨白蛋白-胆红素(ALBI)评分与儿童继发性噬血细胞综合征（sHLH）的临床相关性及其预后价值。.
方法: 回顾性分析2012年1月至2023年3月遵义医科大学附属医院明确诊断的儿童sHLH病例资料，根据ALBI分级进行生存分析，对ALBI评分和临床指标进行Spearman相关性分析，通过ROC曲线确定ALBI评分的最佳截断值并评估其对预后预测的准确性，Kaplan-Meier法绘制生存曲线，log-rank方法比较组间生存曲线差异。采用Cox回归分析进行预后分析，限制性立方样条曲线计算ALBI评分与sHLH患儿死亡风险的关系。.
结果: 共纳入128例sHLH患儿，中位年龄为38(13.25,84)个月，男性70例(54.69%)，女性58例(45.31%)。ALBI分级的生存分析结果显示，ALBI 3级的HLH患儿生存率明显低于ALBI 1级和2级。Spearman相关性分析结果显示，ALBI评分与脾大、呼吸衰竭、弥散性血管内凝血（DIC）、肺出血、消化道出血、中枢神经系统受累、ALT、AST、TG、LDH、PT、APTT、SF呈正相关( r =0.181、0.362、0.332、0.221、0.351、0.347、0.391、0.563、0.180、0.448、0.483、0.370、0.356)，与HB、PLT、FIB呈负相关( r =-0.321、-0.316、-0.423)，与EBV感染、真菌感染、肝大、ANC无显著相关性(P ＞0.05)。应用ROC曲线分析确定ALBI的截断值为-1.76，单因素Cox回归分析结果显示，HB <90 g/L、ALT≥80 U/L、AST≥200 U/L、LDH≥1 000 U/L、PT≥20 s、APTT≥40 s、FIB <1.5 g/L、ALBI≥-1.76、合并肺出血、DIC、中枢神经系统受累、消化道出血和未使用血液净化可能为儿童sHLH预后的危险因素（P <0.05）。多因素Cox回归分析结果显示，FIB <1.5 g/L (HR=2.119, 95%CI ：1.028-4.368)、ALBI≥-1.76 (HR=2.452, 95%CI ：1.233-4.875)、中枢神经系统受累(HR=4.674, 95%CI ：2.486-8.789)是影响预后的独立危险因素，而使用血液净化(HR=0.306, 95%CI ：0.153-0.612)为预后的独立保护因素。应用限制性立方样条显示，死亡风险随ALBI评分增加而增加。ALBI评分对1、2和4周及总体死亡风险预测的ROC曲线下面积（AUC）分别为0.825、0.807、0.700、0.693，对早期死亡风险预测较好。 在年龄≤2岁、合并EBV感染、经HLH-1994/2004治疗、合并呼吸衰竭、ANC≤1.0×109/L、HB＜90 g/L、PLT＜100×109/L、TG≥3.0 mmol/L、LDH≥1 000 U/L、APTT≥40 s、FIB <1.5 g/L的亚组中，与ALBI <-1.76组相比，ALBI≥-1.76 的预后更差(P <0.05)。.
结论: ALBI评分与sHLH的临床特征及实验室指标相关，可作为儿童sHLH预后风险评估的有用指标，对预测儿童sHLH的预后具有较好的准确性和临床应用价值。.
Keywords: hemophagocytic lymphohistiocytosis; ALBI score; children; clinical characteristics; prognosis.