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KMID : 0939920230550031053
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대한암학회지
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-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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KEYWORD
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Erdheim-Chester disease, Non-langerhans cell histiocytosis, Interferon-alpha, Pegylated-interferon α-2a, Ropegylated-interferon α-2b
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