Department of Internal Medicine, Hyogo Prefectural Tsukaguchi Hospital, Japan.
Int Immunopharmacol. 2013 Aug;16(4):488-91. doi: 10.1016/j.intimp.2013.04.033. Epub 2013 May 10.
Lymphocytic variant hypereosinophilic syndromes (L-HES) is thought to be caused by the over-production of interleukin (IL)-5 by type 2 helper cells, which leads to reactive eosinophil expansion and activation. Here we demonstrate the effect of cyclosporine in a patient with L-HES. In the present case, the surface markers of cells from resected lymph nodes or peripheral blood were analyzed by flow cytometry. Serum concentrations of IL-4, IL-5, and IL-8 were measured using an enzyme-linked immunosorbent assay. Methyl-prednisolone pulse therapy followed by the administration of 150 mg/day of cyclosporine combined with 15 mg/day of prednisolone ameliorated eosinophilia. However, abnormal CD3-CD4+ T cell clones remained even when the eosinophil count recovered to normal levels. An elevated IL-8 level was observed only when eosinophils increased. On the other hand, serum IL-4 and IL-5 levels were under detectable limits during the course. Cyclosporine was effective in decreasing the eosinophil count without the elimination of abnormal T cell clones in the present case. Cytotoxic agents may be necessary to cure this serious disease. Moreover, target therapy for IL-8 may be a new strategy for L-HES with high IL-8 and low IL-5 concentrations.
淋巴细胞变异型高嗜酸性粒细胞综合征（L-HES）被认为是由 2 型辅助细胞过度产生白细胞介素（IL）-5 引起的，这导致反应性嗜酸性粒细胞扩增和激活。在这里，我们展示了环孢素在 L-HES 患者中的作用。在本病例中，通过流式细胞术分析了切除的淋巴结或外周血细胞的表面标志物。使用酶联免疫吸附试验测量血清中 IL-4、IL-5 和 IL-8 的浓度。甲基强的松龙脉冲治疗后，给予环孢素 150mg/天联合泼尼松龙 15mg/天，可改善嗜酸性粒细胞增多症。然而，即使嗜酸性粒细胞计数恢复正常，异常的 CD3-CD4+T 细胞克隆仍然存在。仅当嗜酸性粒细胞增加时，才观察到升高的 IL-8 水平。另一方面，在整个过程中，血清 IL-4 和 IL-5 水平低于检测限。在本病例中，环孢素可有效降低嗜酸性粒细胞计数，而不会消除异常 T 细胞克隆。细胞毒性药物可能是治疗这种严重疾病的必要手段。此外，针对 IL-8 的靶向治疗可能是一种新策略，适用于 IL-8 浓度高而 IL-5 浓度低的 L-HES。
