Hypercytokinemia in familial hemophagocytic lymphohistiocytosis.
Hypercytokinemia in familial hemophagocytic lymphohistiocytosis.
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DOI：
10.1182/blood.v78.11.2918.bloodjournal78112918
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发表时间：
1991-12
期刊：
影响因子：
20.3
通讯作者：
Jan-lnge Henter;G. Elinder;Olle Soder;Mona Hansson;Birger Andersson;U. Andersson
中科院分区：
文献类型：
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作者：
Jan-lnge Henter;G. Elinder;Olle Soder;Mona Hansson;Birger Andersson;U. Andersson
家族性噬血细胞性淋巴组织细胞增生症（FHL）是一种经常被忽视，几乎一致致命的儿童疾病。其特征为发热、肝脾肿大、血细胞减少、凝血病和高胆红素血症。FHL的发病机制尚不清楚，但上述临床和实验室结果与几种炎性细胞因子的体外和体内效应报告一致。我们测量了9例FHL患儿的循环干扰素-γ（IFN-γ）、肿瘤坏死因子/恶病质素（TNF）和白细胞介素-6（IL-6）。在活动性疾病期间，七名儿童中有七名检测到IFN-γ升高，六名儿童中有六名检测到TNF升高，六名儿童中有两名检测到IL-6升高。因此，重要的炎性细胞因子在活动性FHL中增加，并可能有助于疾病的发病机制。7名儿童中有7名可溶性CD 8也增加，这表明细胞毒性T淋巴细胞的病理生理重要性。由于FHL似乎与全身性高细胞因子血症有关，我们的研究结果也表明，FHL的研究可能有助于了解细胞因子在体内的作用。此外，FHL是一种遗传性疾病，表明高细胞因子血症是由细胞因子调节的遗传缺陷引起的。
Familial hemophagocytic lymphohistiocytosis (FHL) is a frequently missed and almost uniformly fatal childhood disorder. It is characterized by fever, hepatosplenomegaly, cytopenia, coagulopathy, and hypertriglyceridemia. The pathogenesis of FHL is not known but the above clinical and laboratory findings are compatible with reported in vitro and in vivo effects of several inflammatory cytokines. We measured circulating interferon-gamma (IFN-gamma), tumor necrosis factor/cachectin (TNF), and interleukin-6 (IL-6) in nine children with FHL. During active disease, elevated IFN-gamma was detected in seven of seven children, TNF in six of six, and IL-6 in two of six children studied. Thus, important inflammatory cytokines are augmented in active FHL and may contribute to the pathogenesis of the disease. Soluble CD8 was also increased in seven of seven children, which suggests a pathophysiologic importance of cytotoxic T lymphocytes. Because FHL appears to be associated with a systemic hypercytokinemia, our results also indicate that studies of FHL may contribute to the understanding of cytokine effects in vivo. Moreover, FHL is a hereditary disorder, suggesting that the hypercytokinemia is caused by a genetic defect in cytokine regulation.