Langerhans cell histiocytosis: an update

被引:0
作者
Mataix, J. [1 ]
Betlloch, I. [1 ]
机构
[1] Hosp Gen Alicante, Serv Dermatol, Dept Dermatol, Alicante 03010, Spain
来源
GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA | 2009年 / 144卷 / 02期
关键词
Dendritic cells; Histiocitosis; Langerhans cell; Review; EPSTEIN-BARR-VIRUS; SCLEROSING CHOLANGITIS; LIVER-TRANSPLANTATION; RADIOLOGICAL FEATURES; SECONDARY LEUKEMIA; IMMUNE-SYSTEM; SINGLE-SYSTEM; PULMONARY; DISEASE; ADULT;
D O I
暂无
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
The histiocytoses represent a hetereogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes within various tissues. Langerhans cell histiocytosis (LCH) is the commonest of these disorders and it is associated with high morbidity and mortality, especially in children. LCH is a poorly understood disease with features suggestive of a neoplastic, reactive, or immune dysregulation process. The clinical spectrum of LCH is considered to be broad and includes from self-resolving involvement of a single organ to a potentially fatal multisystem disease. The purpose of this review is to undertake an update of LCH with emphasis on the current recommendations regarding the classification, evaluation and treatment of this enigmatic disease.
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页码:119 / 134
页数:16
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