[Hemophagocytic Lymphohistiocytosis: Clinical Characteristics and Diagnostic Prediction Model]

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1594-1600. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.045.
[Article in Chinese]

Abstract

Objective: To investigate the clinical characteristics of patients with hemophagocytic lymphohistiocytosis (HLH) and quantify the diagnostic value of various indexes in patients with elevated soluble interleukin-2 receptor (sCD25), so as to construct a diagnostic prediction model of HLH.

Methods: The clinical characteristics of 121 patients with elevated sCD25 (≥2 400 U/ml) in the Third Affiliated Hospital of Sun Yat-Sen University were analyzed retrospectively. The patients were divided into HLH group and non-HLH group according to the diagnostic criteria of HLH. The patients with HLH were divided into infection group, tumor group, macrophage activation syndrome (MAS) group and unknown etiology group according to their etiology. The basic data and treatment of the patients were collected for univariate and multivariate logistic analysis to establish a diagnostic prediction model of HLH.

Results: Among the 121 enrolled patients with elevated sCD25, 68 were diagnosed as HLH. The proportion of patients using vasopressors, the incidence rate of disseminated intravascular coagulation (DIC), and the HScore in the HLH group were higher than those in the non-HLH group (P < 0.05). Hepatomegaly, splenomegaly, and hemophagocytosis were more common in HLH patients(P < 0.05). Compared with the patients in non-HLH group, patients in HLH group had lower levels of neutrophils, platelets, fibrinogen, IgG, and IgM, while the levels of triglycerides, ferritin (FER), sCD25, serum glutamic oxaloacetic transaminase (SGOT), alkaline phosphatase (ALP), total bilirubin (TBil), lactate dehydrogenase (LDH), and D-dimer were higher (P < 0.05). In subgroup analysis, the level of sCD25 in tumor group was higher than that in infection group. The level of sCD25/ferritin in tumor group was higher than that in infection group and MAS group. Compared with HLH patients in the tumor group, the procalcitonin (PCT) level, proportion of patients using vasopressors, positive rate of hemophagocytosis, and incidence rate of DIC were all higher in the infection group, and the differences were statistically significant (P < 0.05). The results of multivariate analysis showed that fever, splenomegaly, hemophagocytosis, cytopenias, IgM, M.sCD25 [multiple of sCD25 detection value relative to the diagnostic threshold (2400 U/ml)], fibrinogen, and triglycerides were independent predictive factors for HLH (P < 0.05).The diagnostic prediction model H constructed based on temperature, splenomegaly, hemophagocytosis, cytopenias, IgM, M.sCD25, fibrinogen, triglycerides showed good predictive accuracy. The optimal cutoff value of H was 39.45, the sensitivity of the model was 94.12%, the specificity was 83.02%.

Conclusion: sCD25, sCD25/FER, PCT, hemophagocytosis, hemodynamic instability and DIC could help to distinguish the underlying etiology of HLH. The prediction model H has high discrimination and calibration, which could be used as a relatively accurate clinical diagnostic tool for HLH.

题目: 噬血细胞综合征的临床特征分析及诊断预测模型的构建.

目的: 探讨噬血细胞综合征(HLH)患者的临床特征,量化各项指标在可溶性白细胞介素-2受体(sCD25)升高患者人群中的诊断价值,从而构建HLH诊断预测模型。.

方法: 回顾性分析中山大学附属第三医院121例sCD25升高(≥2 400 U/ml)患者的临床特征。按照是否满足HLH诊断分为HLH组与非HLH组。HLH患者按照病因分为感染组、肿瘤组、巨噬细胞活化综合征(MAS)组、病因不详组。收集患者的基础资料、治疗情况等指标进行单因素及多因素logistic分析,并建立HLH诊断预测模型。.

结果: 121例sCD25升高的患者中68例诊断为HLH。HLH组患者使用升压药物比例、DIC发生比例、HScore评分高于非HLH组(P < 0.05);肝大、脾大、噬血现象在HLH患者中更为常见(P < 0.05);HLH组患者的中性粒细胞、血小板、纤维蛋白原、免疫球蛋白G(IgG)、免疫球蛋白M(IgM)水平更低,而甘油三酯、铁蛋白(FER)、sCD25、血清谷草转氨酶(SGOT)、碱性磷酸酶(ALP)、总胆红素(TBil)、乳酸脱氢酶(LDH)、D-二聚体水平则更高(P < 0.05)。亚组分析结果显示,肿瘤组HLH患者sCD25水平较感染组水平更高、sCD25/FER水平较感染组和MAS组更高;相比于肿瘤组HLH患者,感染组PCT水平、使用升压药物比例、噬血现象阳性率、弥散性血管内凝血(DIC)发生率更高,差异均有统计学意义(P < 0.05)。多因素分析结果提示,发热、脾大、噬血现象、多系减少、IgM、M.sCD25[sCD25检测值相较诊断界值(2 400 U/ml)的倍数]、纤维蛋白原、甘油三酯是HLH的独立预测因素(P < 0.05)。以发热、脾大、噬血现象、多系减少、免疫球蛋白M、M.sCD25、纤维蛋白原、甘油三酯构建的预测模型H显示出较好的预测准确度,当以H=39.45为最佳截断值时,模型的敏感度为94.12%,特异度为83.02%。.

结论: sCD25、sCD25/FER、PCT、噬血现象、血流动力学不稳定、DIC有助于区分HLH的潜在病因。预测模型H具有良好的区分度、校准度,可以作为临床上一个相对准确的HLH诊断工具。.

Keywords: hemophagocytic lymphohistiocytosis; sCD25; HLH-2004; HScore; model construction.

Publication types

  • English Abstract

MeSH terms

  • Female
  • Fibrin Fibrinogen Degradation Products
  • Humans
  • Lymphohistiocytosis, Hemophagocytic* / diagnosis
  • Male
  • Receptors, Interleukin-2 / blood
  • Retrospective Studies

Substances

  • Receptors, Interleukin-2
  • fibrin fragment D
  • Fibrin Fibrinogen Degradation Products