Erdheim-Chester Disease: Case Report
10.12376/j.issn.2097-0501.2022.01.011
- VernacularTitle:Erdheim-Chester病一例
- Author:
Xiaoyuan FAN
1
,
2
;
Ting LIU
2
,
3
;
Hui YOU
1
,
2
;
Jian SUN
2
,
4
;
Xinxin CAO
2
,
3
;
Feng FENG
1
,
2
,
5
;
Fengdan WANG
1
,
2
Author Information
1. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &
2. Peking Union Medical College, Beijing 100730, China.
3. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &
4. Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &
5. Peking Union Medical College, Beijing 100730, China.State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &
- Publication Type:Journal Article
- Keywords:
Erdheim-Chester disease;
histiocytosis;
magnetic resonance imaging
- From:
JOURNAL OF RARE DISEASES
2022;1(1):68-71
- CountryChina
- Language:Chinese
-
Abstract:
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by the infiltration of bone and multiple organs by foamy lipid-laden histiocytes. ECD is easy to be misdiagnosed due to its complicated clinical manifestations. We report a patient who visited the hospital due to hypothalamic dysfunction, with central nervous system, pancreas, and lower limb bones involvements. Together with the evidence of clinical manifestations, imaging and pathology, this patient was diagnosed with ECD. After treatment with interferon-α, both the clinical symptoms and imaging manifestations of this patient were significantly improved.
