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Case Report
- 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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Citations
Citations to this article as recorded by
- An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement
Atsushi Matsunashi, Wang Zhipeng, Akihiko Sugimoto, Masakazu Fujimoto, Akihiko Yoshizawa, Ryo Sakamoto, Michihiro Uyama, Kohei Ikezoe, Kiminobu Tanizawa, Tomohiro Handa, Toyohiro Hirai
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
Naunyn-Schmiedeberg's Archives of Pharmacology.2025;[Epub] CrossRef - Erdheim–Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances
Rishabh Chaudhary, Anand Kumar, Alpana Singh, Vipul Agarwal, Mujeeba Rehman, Arjun Singh Kaushik, Siddhi Srivastava, Sukriti Srivastava, Vikas Mishra
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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Original Article
- Therapeutic Implication of Intracavitary Instillation of Interferon-alpha-2b in Advanced Solid Tumors
- Yeul Hong Kim
- J Korean Cancer Assoc. 1994;26(3):510-519.
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Abstract
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- Malignant pleural effusions and ascites are distressing complications in solid cancer patients with considerable management problems. Intracavitary immunotherapy has been studied recently in a variety of solid tumors that otherwise might be incurable. Using the intraperitoneal administration of a cytokine, such as the interferons and the interleukins, some complete regressions of tumors have been documented. But those trials were applied for malignant lesions that are isolated to the peritoneal cavity, such as residual ovarian cancer. To evaluate the clinical implications of intracavitary interferon-alpha-2b(IFN-a2b) instillation with or without systemic chemotherapy in various solid tumors, thirteen patients (five with gastric, four with hepatic, two with bronchial, one with ovarian, and one with pancreatic tumor) with carcinoma- tous pleural effusions or ascites were treated with intracavitary instillation of IFN-a2b 6MU or 10MU on day 1, 8, 15, repeated every 28 days. Two patients who had recurrent ascites after ini- tial response, were retreated by same method and total IS cases were evaluable. Ten patients were treated with intracavitary IFN-a2b and systemic chematherapy (six with oral UFT and Leucovorin, two with Etoposide, Ifosfomide, and Cisplatin, one with Cyclophsphamide, Adriamycin, and Cisplatin, one with Etoposide, Leucovorin, and Cisplatin). Nine cases (60%) showed clinical evidence of therapeutic benefit by disaPPearance of malignant ascites or pleural effusion. The response ranged from 8 to 44+ weeks with a median of 12+ weeks. Especially responders treated with intracavitary IFN-a2b and systemic chemotherapy showed long- er duration of response. Fever (8/15) and abdominal pain (4/15) were the most common side effects. Patients who treated with IFN-a2b instil1ation only did not show any significant side effects and leukopenia and mucositis which developed after combined therapy with chemotherapy were not seem to be related with IFN-a2b instillation. These results suggest a role of intracavitary IFN-a2b instillation for control of malignant ascites and pleural effusion in advanced solid tumor. Also minor side effects of intracavitary IFNMb instillation suggest that this treatment can be combined safely with systemic chemotherapy.
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