Bone lesions in Langerhans cell histiocytosis [Skelettbefall bei Langerhanszellhistiozytose]

被引：0
作者：
Grois N. [1 ,2 ]
Minkov M. [1 ]
机构：
[1] LCH-Studienzentrale, Forschungsinstitut für Krebskranke Kinder, St.-Anna-Kinderspital, Wien
[2] Forschungsinstitut für Krebskranke Kinder, 1090 Wien
来源：
Monatsschrift Kinderheilkunde | 2006年 / 154卷 / 1期
关键词：
Bone; Children; Langerhans cell histiocytosis; Skeleton; Treatment;
D O I：
10.1007/s00112-005-1272-5
中图分类号：
学科分类号：
摘要：
Langerhans cell histiocytosis (LCH) is a reactive disorder of the dendritic cell system and is characterized by proliferation of cells bearing the phenotype of the normal Langerhans cells. Bone lesions are the most common manifestation of LCH and account for about 1% of all childhood bone tumors. They occur in about 80% of paediatric cases of LCH, either in the form of isolated bony disease (single system LCH) or within the context of a multisystem disease (multisystem LCH). Images of LCH bone lesions are not pathognomonic, and histopathological confirmation of the diagnosis is therefore mandatory. LCH confined to the skeleton has an excellent prognosis, and only local treatment (curettage, intralesional steroids) is given in most cases. In cases of extended lesions in weight-bearing bones or of multiple bone lesions systemic therapy may be indicated. © Springer Medizin Verlag 2005.
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页码：32 / 40
页数：8
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