静脉注射丙种球蛋白对川崎病外周血单个核细胞CD40L表达的影响

目的：观察川崎病（kawasaki disease,KD)急性期患儿外周血单个核细胞（PBMC）表面CD40配体（CD40L）表达水平，及静脉注射丙种球蛋白（IVIG）治疗后CD40L的变化，以期探讨IVIG治疗机制及冠状动脉病变（CAL）发生的可能机制。方法：用流式细胞技术法检测11例川崎病急性期患儿及1g/kg IVIG治疗后其PBMC表达CD40L阳性细胞率及平均荧光强度（MFI）。结果：川崎病患者PBMC表面CD40L表面较正常对照持久；对1g/kg IVIG敏感者治疗后患者PBMC上表达CD40L下降，与治疗前比较有显著性差异；对1g/kg IVIG不敏感者治疗后PBMC中高表达CD40L的细胞数增多，与正常对照比较有显著性差异；2例急性期已发生冠状动脉扩张的患儿其PBMC上的CD40L表达的阳性细胞率及平均荧光强度均明显高于无冠状动脉病变者。结论：CD40L升高在川崎病发病及冠状动脉病变中起一定作用，IVIG可能通过下调川崎病PBMC上CD40L表达水平预防川崎病冠状动脉病变的发生。

The effect of IVIG on expression of CD40L on PBMC in Kawasaki disease patients

Objective To observe the levels of CD40 ligand (CD40L) in peripheral blood mononuclear cells (PBMC) in Kawasaki disease (KD) patients, and explore the mechanism of intravenous immunoglobulin (IVIG) in the treatment of KD. Methods CD40L expression on PBMC was detected by flow cytometry in 11 KD patients pre and post IVIG and 13 normal children. Results The expression of CD40L in KD patients was prolonged compared with normal control PBMC. In patients sensitive to IVIG the mean fluorescence intensity (MFI) of positive cells for CD40L was significantly lower compared post IVIG with pre IVIG. The percentage of cells positive for CD40L was significantly higher compared post IVIG with normal control in patients insensitive to IVIG. The expression of CD40L in 2 KD patients with coronary artery lesion was higher than other KD patients. Conclusions Higher levels of CD40L expression may play a very important role in the pathogenesis of Kawasaki disease and coronary artery lesion. The beneficial effect of IVIG in the treatment of KD may be due to the ability of IVIG to down regulate the expression of CD40L on PBMC.