Chehimi J, Elder M, Greene J, Noroski L, Stiehm E R, Winkelstein J A, Sullivan K E
The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
Clin Immunol. 2001 Jul;100(1):49-56. doi: 10.1006/clim.2001.5039.
Hyper-IgE syndrome is characterized by severe recurrent staphylococcal infections, eczema, bone abnormalities, and markedly elevated levels of immunoglobulin E (IgE). The genetic basis is not known and the central immunologic defect is largely undefined. Reduced neutrophil chemotaxis is often described, and variable T cell defects have been demonstrated in some patients. It has been hypothesized that hyper-IgE is associated with a Th1/Th2 imbalance. We wished to characterize cytokine and chemokine imbalances that might reflect the underlying disease process or reflect ongoing pathologic processes. Nine patients with hyper-IgE syndrome and six controls were studied. Radioimmunoassays, flow cytometry, and gene array analyses were performed to characterize cytokine and chemokine production. Hyper-IgE patients express more IL-12, while ENA-78, MCP-3, and eotaxin are markedly underexpressed. Underexpression of a set of chemokines could explain a number of features of hyper-IgE syndrome and may offer a new paradigm for the understanding of this disorder.
高免疫球蛋白E综合征的特征为严重复发性葡萄球菌感染、湿疹、骨骼异常以及免疫球蛋白E（IgE）水平显著升高。其遗传基础尚不清楚，主要免疫缺陷也大多未明确。常描述有中性粒细胞趋化性降低，且在部分患者中已证实存在不同的T细胞缺陷。据推测，高IgE与Th1/Th2失衡有关。我们希望明确可能反映潜在疾病过程或正在进行的病理过程的细胞因子和趋化因子失衡情况。对9例高免疫球蛋白E综合征患者和6名对照者进行了研究。采用放射免疫测定、流式细胞术和基因阵列分析来明确细胞因子和趋化因子的产生情况。高IgE患者表达更多的白细胞介素-12，而ENA-78、单核细胞趋化蛋白-3和嗜酸性粒细胞趋化因子表达明显不足。一组趋化因子的表达不足可以解释高免疫球蛋白E综合征的一些特征，并可能为理解这种疾病提供新的范例。
