| Case Report of Erdheim-Chester Disease Successfully Treated with Pegylated Interferon: A Single-Center Experience |
Yujin Lim1
, Sang Eun Yoon2, Junhun Cho3, Darae Kim4, Chul Won Jung2
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1Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
2Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
3Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
4Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
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Received: November 21, 2022; Accepted: January 18, 2023. Published online: January 19, 2023.
*Yujin Lim and Sang Eun Yoon contributed equally to this work. |
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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-alpha (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. |
| Key words:
Erdheim–chester disease, Non-langerhans cell histiocytosis, Interferon-alpha, Pegylated-interferon α-2a, Ropegylated-interferon α-2b |
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