[1]张 鼎,赵 斌.朗格汉斯组织细胞增生症的诊治进展[J].医学信息,2019,32(19):35-38.[doi:10.3969/j.issn.1006-1959.2019.19.012]
 ZHANG Ding,ZHAO Bin.Progress in the Diagnosis and Treatment of Langerhans Cell Histiocytosis[J].Journal of Medical Information,2019,32(19):35-38.[doi:10.3969/j.issn.1006-1959.2019.19.012]
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朗格汉斯组织细胞增生症的诊治进展()
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医学信息[ISSN:1006-1959/CN:61-1278/R]

卷:
32卷
期数:
2019年19期
页码:
35-38
栏目:
综述
出版日期:
2019-10-01

文章信息/Info

Title:
Progress in the Diagnosis and Treatment of Langerhans Cell Histiocytosis
文章编号:
1006-1959(2019)19-0035-04
作者:
张 鼎1赵 斌2
(1.福建中医药大学,福建 福州 350108;2.厦门市中医院,福建 厦门 361009)
Author(s):
ZHANG Ding1ZHAO Bin2
(1.Fujian University of Traditional Chinese Medicine,Fuzhou 350108,Fujian,China;2.Xiamen Hospital of Traditional Chinese Medicine,Xiamen361009,Fujian,China)
关键词:
朗格汉斯组织细胞增生症单核-巨噬细胞异常增生炎性髓样肿瘤
Keywords:
Langerhans cell histiocytosisMononuclear-macrophage dysplasiaInflammatory myeloid tumor
分类号:
R55
DOI:
10.3969/j.issn.1006-1959.2019.19.012
文献标志码:
A
摘要:
朗格汉斯组织细胞增生症(LCH)是一种少见的、病因尚不明确的单核-巨噬细胞异常增生性疾病,其临床表现具有多样性,累及多个系统,且发病机制复杂,目前主要认为是炎性髓样肿瘤,治疗上仍以联合化疗为主,随着靶向药物及骨髓移植应用逐渐增多,其并发症及不良反应需进一步解决。对于单系统受累LCH 患者预后较佳,临床报道治愈病例较多。而多系统受累LCH患者预后不佳,病死率高。本文就近年来有关LCH的发病机制、临床表现、诊断与治疗及预后进展进行综述。
Abstract:
Langerhans cell histiocytosis (LCH) is a rare mononuclear-macrophage dysplasia with unclear etiology. Its clinical manifestations are diverse, involving multiple systems, and the pathogenesis is complex. At present, it is mainly considered to be an inflammatory myeloid tumor. The treatment is still based on combined chemotherapy. With the increasing application of targeted drugs and bone marrow transplantation, its complications and adverse reactions need to be further resolved. The prognosis of LCH patients with single system involvement is better, and more cases are reported in clinical reports. The multi-system involvement of LCH patients has a poor prognosis and a high mortality rate. This article reviews recent advances in the pathogenesis, clinical manifestations, diagnosis and treatment, and prognosis of LCH.

参考文献/References:

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更新日期/Last Update: 2019-10-01