Pre-B-cell colony-enhancing factor is markedly elevated in childhood hemophagocytic lymphohistiocytosis
Pre-B-cell colony-enhancing factor is markedly elevated in childhood hemophagocytic lymphohistiocytosis
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儿童噬血细胞性淋巴组织细胞增多症中前 B 细胞集落增强因子显着升高
DOI：
10.4238/2015.may.18.21
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发表时间：
2015-01-01
影响因子：
0.4
通讯作者：
Lan, D.
中科院分区：
文献类型：
--
作者：
Gao, Z. Y.;Li, X. Y.;Lan, D.
噬血细胞性淋巴组织细胞增多症（HLH）是一种危及生命的综合征，涉及高细胞素血症的最终共同途径，其中肿瘤坏死因子(TNF)- α，干扰素(IFN)- γ和可溶性白细胞介素2受体- α (sIL-2R α)是关键的细胞因子。前b细胞集落增强因子（PBEF）是一种参与多种炎症性疾病的炎症细胞因子。然而，它在HLH中的作用尚不清楚。在这项研究中，我们研究了PBEF在HLH中的作用。收集了22例HLH患儿和14例健康患儿的血浆。采用酶联免疫吸附法测定血浆PBEF、tnf - α、ifn - γ和sIL-2R α的浓度。所有临床数据均来源于医疗记录。急性期患儿PBEF、tnf - α、ifn - γ、sIL2-R α水平明显高于健康儿童（P < 0.05）。治疗后13例患儿病情好转，PBEF、tnf - α、ifn - γ水平降至正常水平（P < 0.05）；sIL-2R α水平也有所下降（P < 0.05），但仍高于正常水平（P < 0.05）。2例患者失访，7例患者治疗反应不良最终死亡，PBEF水平高于幸存者（P < 0.01）。PBEF水平与tnf - α、ifn - γ、sIL-2R α、血清铁蛋白、甘油三酯呈显著正相关（P < 0.05），与纤维蛋白呈显著负相关（P < 0.05）。PBEF似乎参与了HLH的炎症过程，PBEF的升高与疾病活动性有关。我们目前正在评估PBEF作为诊断和管理患者的标志物的作用。
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome involving a final common pathway of hypercytokinemia, in which tumor necrosis factor (TNF)-alpha, interferon (IFN)-gamma, and soluble interleukin 2-receptor-alpha (sIL-2R alpha) are the key cytokines. Pre-B-cell colony-enhancing factor (PBEF) is an inflammatory cytokine involved in several inflammatory diseases. However, its role in HLH is unknown. In this study, we examined the role of PBEF in HLH. Plasma was collected from 22 children with HLH and 14 healthy children. The concentrations of plasma PBEF, TNF-alpha, IFN-gamma, and sIL-2R alpha were determined using an enzyme-linked immunosorbent assay. All clinical data were derived from medical records. In the acute phase, children with HLH had much higher PBEF, TNF-alpha, IFN-gamma, and sIL2-R alpha levels than did healthy children (P < 0.05). After treatment, 13 HLH children improved and PBEF, TNF-alpha, and IFN-gamma levels decreased to normal levels (P < 0.05); sIL-2R alpha levels also decreased (P < 0.05), but remained above the normal level (P < 0.05). Two patients were lost to follow-up, while 7 patients showed a bad response to therapy and eventually died, showing high PBEF levels above those of the survivors (P < 0.01). PBEF level was significantly positively correlated with TNF-alpha, IFN-gamma, sIL-2R alpha, serum ferritin, and triglycerides (all P < 0.05), and was negatively correlated with fibrin (P < 0.05). PBEF appears to be involved in the inflammatory process of HLH, and elevated PBEF is related to disease activity. We are currently evaluating the role of PBEF as a marker for the diagnosis and management of patients.