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- Case Report of Erdheim-Chester Disease Successfully Treated with Pegylated Interferon: A Single-Center Experience
- Yujin Lim, Sang Eun Yoon, Junhun Cho, Darae Kim, Chul Won Jung
- Cancer Res Treat. 2023;55(3):1053-1057. Published online January 19, 2023
- DOI: https://doi.org/10.4143/crt.2022.1535
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Abstract
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ePub - Erdheim-Chester disease (ECD), also known as non-Langerhans cell histiocytosis, is a multi-systemic disease with unclear pathogenesis. Based on a small number of case studies, pegylated interferon-α (PEG-IFN-α) has been used as the front-line treatment option. However, there are limited data regarding administration of ropegylated-interferon α-2b (ROPEG-IFN-α 2b) for ECD patients. Herein, we report two cases of severe ECD treated with two types of PEG-IFN-α. One patient with heart and skeleton involvement and BRAF V600E mutation was treated with weekly PEG-IFN-α 2a. Another patient with bone involvement and no BRAF V600E mutation was administered monthly ROPEG-IFN-α 2b. The two types of PEG-IFN-α showed excellent disease control, excellent survival outcomes, and manageable toxicities in ECD patients. These results suggest that ROPEG-IFN-α 2b could be used equivalently to PEG-IFN-α 2a for management of advanced ECD.
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- An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement
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Internal Medicine.2025;[Epub] CrossRef - Recent advances in therapeutic strategies of Erdheim-Chester disease
Rohit Doke, Rahul Lokhande, Kalyani Chande, Kuldeep Vinchurkar, Bhupendra G. Prajapati
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Disease-a-Month.2025; 71(2): 101845. CrossRef - Advances in Understanding and Management of Erdheim-Chester Disease
Aniruddha Murahar Kulkarni, Prasanna Kumar Reddy Gayam, Jesil Mathew Aranjani
Life Sciences.2024; 348: 122692. CrossRef
- An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement
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