KMID : 0939920230550031053
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´ëÇѾÏÇÐȸÁö 2023 Volume.55 No. 3 p.1053 ~ p.1057
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Case Report of Erdheim-Chester Disease Successfully Treated with Pegylated Interferon: A Single-Center Experience
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Lim Yu-Jin
Yoon Sang-Eun Cho Jun-Hun Kim Da-Rae Jung Chul-Won
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Abstract
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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-¥á (PEG-IFN-¥á) has been used as the front-line treatment option. However, there are limited data regarding administration of ropegylated-interferon ¥á-2b (ROPEG-IFN-¥á 2b) for ECD patients. Herein, we report two cases of severe ECD treated with two types of PEG-IFN-¥á. One patient with heart and skeleton involvement and BRAF V600E mutation was treated with weekly PEG-IFN-¥á 2a. Another patient with bone involvement and no BRAF V600E mutation was administered monthly ROPEG-IFN-¥á 2b. The two types of PEG-IFN-¥á showed excellent disease control, excellent survival outcomes, and manageable toxicities in ECD patients. These results suggest that ROPEG-IFN-¥á 2b could be used equivalently to PEG-IFN-¥á 2a for management of advanced ECD.
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KEYWORD
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Erdheim-Chester disease, Non-langerhans cell histiocytosis, Interferon-alpha, Pegylated-interferon ¥á-2a, Ropegylated-interferon ¥á-2b
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