Associations between inflammatory cytokines and organ damage in pediatric patients with hemophagocytic lymphohistiocytosis
Associations between inflammatory cytokines and organ damage in pediatric patients with hemophagocytic lymphohistiocytosis
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噬血细胞性淋巴组织细胞增多症儿科患者炎症细胞因子与器官损伤的关系
DOI：
10.1016/j.cyto.2016.05.022
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发表时间：
2016-09-01
期刊：
影响因子：
3.8
通讯作者：
Shen, Di-Ying
中科院分区：
文献类型：
--
作者：
Yang, Shi-Long;Xu, Xiao-Jun;Shen, Di-Ying
噬血细胞淋巴组织细胞增多症(HLH)是一种潜在的致命性疾病，其特征是强烈的炎症反应和多器官损害。大多数临床和实验室表现被认为与高细胞分裂素血症和淋巴细胞和组织细胞的器官浸润有关。本研究的目的是探讨细胞因子与人促黄体生成素的不同表现之间的关系。在这项回顾性研究中，共有105名被诊断为HLH的患者入选。收集的信息包括患者的人口学特征、临床和实验室结果以及细胞因子数据。确诊时的中位年龄为2.8岁，74名患者(70.4%)记录有EB病毒感染。半数以上患者出现肝大(88.6%)、脾大(81.9%)、细胞减少(68.6%)、铁蛋白升高(93.3%)、低纤维蛋白原血症(61.9%)和吞噬血细胞(77.3%)。IL-6、IL-10和干扰素-γ在大多数患者中呈中度或显著升高。在相关分析中，干扰素-γ与丙氨酸氨基转移酶(ALT)、天冬氨酸氨基转移酶(AST)、胆红素、乳酸脱水酶(LDH)、甘油三酯、纤维蛋白原密切相关，IL-10与血小板计数相关。根据细胞因子水平将患者分为两组，干扰素-γ水平高的患者ALT、AST、胆红素、乳酸脱氢酶、甘油三酯和纤维蛋白原水平较高，而IL-10水平较高的患者的血红蛋白和血小板计数明显降低。综上所述，本研究提出了高细胞分裂素血症与HLH器官损害有关的临床证据。干扰素-γ可能与肝脏损害和凝血系统疾病有关，而IL-10是与细胞减少症相关的细胞因子。(C)2016爱思唯尔有限公司。保留所有权利。
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease characterized by overwhelming inflammation response and multiple organ damage. Most of the clinical and laboratory manifestations of HLH are thought to be related to hypercytokinemia and organ infiltration with lymphocytes and histiocytes. The aim of this study was to investigate the associations between cytokines and various manifestations of HLH. A total of 105 patients diagnosed with HLH were enrolled in this retrospective study. The information including the patients' demographic characteristics, clinical and laboratory findings at presentation and cytokine data were collected. The median age at diagnosis was 2.8 years, with 74 patients (70.4%) documented Epstein-Barr virus infection. Hepatomegaly (88.6%), splenomegaly (81.9%), cytopenia (68.6%), elevated ferritin level (93.3%), hypofibrinogenemia (61.9%) and hemophagocytosis (77.3%) were found in more than half of the patients. Interleukin (IL)-6, IL-10 and interferon (IFN)-gamma were found to be moderately or significantly elevated in most patients. In the correlation analysis, IFN-gamma was closely related to the concentration of alanine aminotransferase (ALT), aspartate aminotransferase (AST), bilirubin, lactate dehydrase (LDH), triglyceride and fibrinogen, while IL-10 was associated with platelet count. When split the patients into two groups according to the cytokine levels, patients with high IFN-gamma presented higher level of ALT, AST, bilirubin, LDH, triglyceride, and fibrinogen, while patients with high IL-10 presented much lower hemoglobin and platelet count. In conclusion, the present study put forward clinical evidence that hypercytokinemia is related to organ damage in HLH. IFN-gamma may contribute to liver impairment and coagulation disease, while IL-10 is a cytokine related to cytopenias. (C) 2016 Elsevier Ltd. All rights reserved.