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Cyclosporine A therapy for multisystem langerhans cell histiocytosis.

机译:环孢霉素A一种用于治疗多系统Langerhans细胞组织细胞增多症的疗法。

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BACKGROUND: Treatment of multisystem Langerhans cell histiocytosis (LCH) remains difficult. Various regimens of single and multiagent chemotherapy have been used, but a significant proportion of patients fail to respond to treatment. PROCEDURE: We have evaluated the use of cyclosporine A (CSA) in a controlled group of patients, who had received a systematic primary therapy (LCH-I). Patients received CSA either as a single agent (10 patients) or in combination with vinblastine, etoposide, prednisolone, and/or antithymocyte globulin (16 patients). RESULTS: Among the total of 26 patients treated, a single patient developed a complete response and three a partial response, whereas 85% (22 patients) had no response to CSA. CONCLUSIONS: CSA is at best of limited value in the treatment of patients with multisystem LCH, particularly those who had progressive disease while receiving chemotherapy. Copyright 1999 Wiley-Liss, Inc.
机译:背景:多系统朗格汉斯细胞组织细胞增生症(LCH)的治疗仍然很困难。已经使用了多种单药和多药化疗方案,但是很大一部分患者对治疗无效。程序:我们评估了环孢霉素A(CSA)在接受系统性主要治疗(LCH-1)的对照组患者中的使用。患者接受CSA单一治疗(10例),或与长春碱,依托泊苷,泼尼松龙和/或抗胸腺细胞球蛋白联合治疗(16例)。结果:在总共接受治疗的26位患者中,有1位患者出现了完全缓解,有3位患者出现了部分缓解,而85%(22位患者)对CSA没有缓解。结论:对于多系统LCH患者,尤其是在接受化疗的同时进行性疾病的患者,CSA充其量是有限的。版权所有1999 Wiley-Liss,Inc.

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