Cancer Res Treat.  2023 Jul;55(3):1053-1057. 10.4143/crt.2022.1535.

Case Report of Erdheim-Chester Disease Successfully Treated with Pegylated Interferon: A Single-Center Experience

• Lim Y
• Yoon SE
• Cho J
• Kim D
• Jung CW

• KMID: 2544185
• DOI: http://doi.org/10.4143/crt.2022.1535

Abstract

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.

Keyword

Erdheim-Chester disease; Non-langerhans cell histiocytosis; Interferon-alpha; Pegylated-interferon α-2a; Ropegylated-interferon α-2b

Figure

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  Fig. 1 (A) Chest X-ray at diagnosis shows bilateral pleural effusion and pulmonary edema. (B) Skeletal computed tomography shows osteolytic lesion at right ilium and bilateral symmetric osteosclerosis of both distal femurs and proximal and distal tibia. (C) Heart biopsy presented proliferation of foamy histiocytes (H&E stain, ×100) (i), positive immunostaining of CD68 (x100) (ii) and CD163 (x100) (iii), but negative immunostaining of CD1a (x100) (iv). (D) The pleural effusion had resolved in the chest X-ray after 3 months of pegylated interferon-α 2a injections.

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  Fig. 2 (A) Baseline positron emission tomography shows multiple hypermetabolic bone lesions and lymphadenopathy. (B) Bone marrow biopsy reveals numerous histiocytic infiltration (H&E stain, ×200) (i), CD68-positive staining (×200) (ii), CD163-positive staining (×200) (iii) and CD1a-negative staining (×200) (iv). (C) After ropegylated-interferon α-2b, the disease nearly resolved in follow-up positron emission tomography.

Reference

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