Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome
Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome
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DOI：
10.1111/j.1365-2141.2008.07298.x
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发表时间：
2008-10-01
影响因子：
6.5
通讯作者：
Luo, Chunfang
中科院分区：
文献类型：
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作者：
Tang, Yongmin;Xu, Xiaojun;Luo, Chunfang
噬血细胞综合征(HPS)是一种罕见但往往致命的免疫调节障碍，由高细胞分裂素血症引起。应用流式细胞仪微珠阵列技术检测24例初治HPS患儿和87例正常对照儿童血清辅助性T细胞1型(Th1)和Th2型(Th2)细胞因子干扰素-γ(IFN-γ)、肿瘤坏死因子(TNF)、白细胞介素10(IL-10)、IL-6、IL-4和IL-2的水平。HPS急性期血清干扰素-γ、IL-10和IL-6的中位数分别为901.7、879.0和63.8pg/ml，显著高于缓解期、健康人和病毒感染者。IL-4在急性期轻度升高，而IL-2和肿瘤坏死因子在正常范围。细菌性脓毒症患者IL-6水平极高，IL-10中等水平，而干扰素-γ仅轻度升高。根据Th1/Th2细胞因子模式，5例患者在符合相关诊断标准前3~13d诊断为HPS。IL-10水平和GT2000pg/ml是影响HPS疗效(P=0.033)和预后(P=0.009)的不良预后因素。我们认为，干扰素-γ和IL-10的显著升高以及IL-6水平的轻微升高是儿童HPS的早期、特异性和预后的细胞因子模式。
The haemophagocytic syndrome (HPS) is a rare but frequently fatal disorder of immune regulation caused by hypercytokinemia. Using cytometric bead array technique, the serum T-helper cell type 1 (Th1) and 2 (Th2) cytokines including interferon-gamma (IFN-gamma), tumour necrosis factor (TNF), interleukin (IL)-10, IL-6, IL-4 and IL-2 were determined in 24 children with de novo HPS and 87 children as control. The median levels of serum IFN-gamma, IL-10 and IL-6 in the acute phase of HPS were 901.7, 879.0 and 63.8 pg/ml, respectively, significantly higher than those after remission, and in the healthy volunteers and patients with viral infection. IL-4 was slightly elevated while IL-2 and TNF were within normal range in acute phase. Patients with bacterial sepsis showed an extremely high level of IL-6 and moderate level of IL-10, whereas IFN-gamma was only slightly elevated. Five patients were diagnosed with HPS according to the Th1/Th2 cytokine pattern 3-13 d earlier than they fulfilled the relevant diagnostic criteria. IL-10 level > 2000 pg/ml was an unfavorable prognostic factor for HPS treatment response (P = 0.033) and outcome (P = 0.009). We conclude that the significant increase of IFN-gamma and IL-10 and a slightly increased level of IL-6 is an early, specific and prognostic cytokine pattern for childhood HPS.