克拉屈滨治疗难治复发朗格汉斯细胞组织细胞增生症1例并文献复习

Salvage treatment of recurrent langerhans cell histiocytosis with a combination of cladribine and cytarabine

目的：观察克拉屈滨联合阿糖胞苷治疗朗格汉斯细胞组织细胞增生症（LCH）的疗效,探讨克拉屈滨治疗 LCH 的机制和适应证范围。方法选取 LCH 使用克拉屈滨联合阿糖胞苷治疗患儿1例,收集临床资料和转归,进行分析。结果经4个疗程克拉屈滨联合阿糖胞苷治疗,LCH 患儿临床症状好转,无疾病活动表现。药物不良反应主要是骨髓抑制,每疗程均发生Ⅲ~Ⅳ级血液系统不良反应。结论克拉屈滨在儿童复发及难治性 LCH 中,是极具潜力的治疗药物之一,对难治复发患者缓解率高,不良反应及远期疗效尚有待观察。但从该药物的作用机制及目前临床经验分析,具有良好前景。

Objective Explore the chemotherapy of recurrent Langerhans cell histiocytosis （LCH） of high risk in children with cladribine and cytarabine. Method Observe the clinical manifestation of one child with relapsed multi-system LCH and the response to 4 courses of cladribine/ cytarabine, and review the related literature. Rsult The pa-tient achieved complete remission and had no sign of disease reactivation for 15 months of follow-up, and her diabe-tes insipidus was improved since the first course of therapy. The main side effect is Ⅳ degree of hematologic toxici-ty. Conclution Cladribine is active and well-tolerated in refractory high risk LCH, though there is still much room for further study.