Successful Treatment of Erdheim-Chester Disease With Coronary Artery Involvement |
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| Authors: | Ai-lin Zhao Yi-ning Wang Feng-Dan Wang Na Niu Jian Sun Yue-ying Mao Dao-bin Zhou Jian Li Xin-xin Cao |
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| Institution: | 1. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China;2. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China;3. Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China;4. Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China |
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| Abstract: | Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis and inflammatory myeloid neoplasm with poor prognosis. Symmetric long bone osteosclerosis occurs in nearly all patients, but other organs are often involved. Coronary artery involvement is rare, but was encountered in a patient who experienced angina. Radiologic presentation and histologic findings were consistent with diagnosis of ECD. A soft-tissue mass was found surrounding the right atrium, ascending aorta, and all branches of coronary artery. Interferon-alfa treatment was successful. In conclusion, we recommend coronary artery computed tomography angiography for cardiovascular evaluation of ECD and interferon-alfa to treat ECD. |
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| Keywords: | Corresponding author: Dr Xin-xin Cao Department of Hematology Peking Union Medical College Hospital 1 Shuai Fu Yuan Hu Tong Dongcheng District Beijing 100730 China Tel : +86-010-69155027 fax: +86-010-63941685 |
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