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朗格汉斯细胞组织细胞增生症治疗进展

  • 泥永安
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  • 青岛大学附属医院儿科(山东青岛 266000)

收稿日期: 2015-03-15

  网络出版日期: 2015-03-15

Progress in the treatment of Langerhans cell histiocytosis

  • NI Yongan
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  • Department of Pediatrics, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong, China

Received date: 2015-03-15

  Online published: 2015-03-15

摘要

目前朗格汉斯细胞组织细胞增生症(LCH)在治疗上采用分型、分级而分治的策略。研究表明,单系统病变(SS-LCH)的预后好,而伴危险器官受累的多系统病变(MS-LCH)及难治性/复发性病例(Re-LCH)预后差。随着化学疗法的进步,伴危险器官受累的MS-LCH 5年生存率已达80%以上,Re-LCH的治疗有效率达60%以上,Re-LCH可行造血干细胞移植以达到根治。

本文引用格式

泥永安 . 朗格汉斯细胞组织细胞增生症治疗进展[J]. 临床儿科杂志, 2015 , 33(3) : 291 . DOI: 10.3969j.issn.1000-3606.2015.03.023

Abstract

Treatment of Langerhans cell histiocytosis (LCH) needs to be tailored for each individual patient according to LCH classification currently. Single-system LCH (SS-LCH) has an excellent prognosis. However, there is a poor prognosis in multisystem LCH (MS-LCH) with risk organs (RO) involvement and refractory or recurrent LCH (Re-LCH). The prognosis of MS-LCH with RO involvement and Re-LCH has been improved markedly accompanying with progress of chemotherapy in recent years. The 5-year survival rate of MS-LCH reached above 80%, and the effective rate of Re-LCH reached above 60% after chemotherapy. Re-LCH can be cured by hematopoietic stem cell transplantation.
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