环孢素A治疗高免疫球蛋白E综合征
Cyclosporin A in hyperimmunoglobulin E syndrome.
作者信息
Etzioni A, Shehadeh N, Brecher A, Yorman S, Pollack S
机构信息
Department of Pediatrics and Clinical Immunology, Rambam Medical Center, Haifa, Israel.
出版信息
Ann Allergy Asthma Immunol. 1997 Apr;78(4):413-4. doi: 10.1016/S1081-1206(10)63204-8.
BACKGROUND
The hyperimmunoglobulinemia E syndrome is a serious disorder of an unknown etiology without effective treatment. Cyclosporin A has immunosuppressive properties. Its effect in a patient with hyperimmunoglobulinemia E syndrome was evaluated.
METHODS
We report a 3-year-old boy with severe hyperimmunoglobulinemia E syndrome in whom various therapeutic modalities were ineffective. He was started on cyclosporin A 3 mg/kg/d for 6 months. We examined several humoral and cellular immune functions during the treatment period and monitored his clinical condition.
RESULTS
Marked improvement in his clinical condition was observed during therapy with cyclosporin A. There was a significant decrease in serum IgE and significant increase in neutrophil chemotactic function. No change in CD40 ligand expression was observed. Cyclosporin A blood level did not exceed 150 ng/mL during the study.
CONCLUSIONS
A small dose of cyclosporin A (3 to 5 mg/kg/d) is beneficial in patients with hyperimmunoglobulinemia E syndrome. It should be considered in severe cases where other therapeutic modalities have failed.
背景
高免疫球蛋白E综合征是一种病因不明的严重疾病，尚无有效治疗方法。环孢素A具有免疫抑制特性。对其在一名高免疫球蛋白E综合征患者中的疗效进行了评估。
方法
我们报告一名3岁患有严重高免疫球蛋白E综合征的男孩，多种治疗方式均无效。开始给予他环孢素A，剂量为3mg/kg/d，持续6个月。在治疗期间，我们检测了多种体液和细胞免疫功能，并监测其临床状况。
结果
在使用环孢素A治疗期间，观察到其临床状况有明显改善。血清IgE显著降低，中性粒细胞趋化功能显著增强。未观察到CD40配体表达有变化。在研究期间，环孢素A血药浓度未超过150ng/mL。
结论
小剂量环孢素A（3至5mg/kg/d）对高免疫球蛋白E综合征患者有益。在其他治疗方式失败的严重病例中应考虑使用。
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