Review
doi: 10.7534/j.issn.1009-2137.2016.04.055.
[Diagnosis and Treatment of Erdheim-Chester Disease -Review]
[Article in
Chinese]
Affiliations
- PMID: 27531811
- DOI: 10.7534/j.issn.1009-2137.2016.04.055
Item in Clipboard
Review
[Diagnosis and Treatment of Erdheim-Chester Disease -Review]
[Article in
Chinese]
Zhongguo Shi Yan Xue Ye Xue Za Zhi.
2016 Aug.
Abstract
Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. The etiology of ECD is unknown yet. The disease is characterized by the infiltration of lipid-laden histiocytes with foamy to bones and various organs. The heterogeneous manifestations of ECD vary among different individuals. This results in a presentation that may vary from an indolent focal disease to a life threatening organ failure. This review focuses on the etiology, clinical presentation, diagnostic criteria and treatment of Erdheim-Chester disease (ECD).
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