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- Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report
- Yeoun Eun Sung, Yoon Seo Lee, Jieun Lee, Kyo Young Lee
- J Pathol Transl Med. 2018;52(3):183-190. Published online December 27, 2017
- DOI: https://doi.org/10.4132/jptm.2017.10.16
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- 224 Download
- 9 Web of Science
- 8 Crossref
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Abstract
PDF - Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis and multisystem disease. First described in 1930, there are no more than 750 cases reported. The etiology remains unknown, but a majority of cases of ECD and Langerhans cell histiocytosis were found to have clonal mutations involving genes of the mitogen-activated protein kinase pathway. We recently encountered a 53-year-old male patient with extensive ECD involving the systemic lymph nodes, pleura, liver, and long bones clinically mimicking malignant lymphoma. Biopsies were performed at multiple sites, including a pleural mass, an external iliac lymph node, bone marrow, and the liver. Based on histopathological and immunohistochemical findings of positivity for CD68 and negativity for CD1a and S-100, the patient was diagnosed with ECD. Interferon-α was administered as the first-line treatment, but the patient rapidly progressed to hepatic failure after 2 months of treatment. We report this rare case of ECD clinically mimicking malignant lymphoma and diagnosed by careful pathological review.
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Citations
Citations to this article as recorded by
- Pathologic characteristics of histiocytic and dendritic cell neoplasms
Sun Och Yoon
Blood Research.2024;[Epub] CrossRef - Erdheim Chester Disease Mimicking Lymphoma: A Case Report
Philipp Moritz Wunschel, Wolfgang Voss, Marc Keberle
RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren.2022; 194(03): 310. CrossRef - Erdheim–Chester Disease with Isolated CNS Involvement: A Systematic Review of the Literature
Anam Haque, Carlos A. Pérez, Thejasvi A. Reddy, Rajesh K. Gupta
Neurology International.2022; 14(3): 716. CrossRef - Erdheim‐Chester disease with long‐standing diabetes insipidus and generalized edema
Faezeh Sadat Naji, Minoo Sadat Hajmiri, Zahra Mazari, Faeze Salahshour, Vahid Soleimani, Manouchehr Nakhjavani, Mahboobeh Hemmatabadi
Clinical Case Reports.2021;[Epub] CrossRef - Morbus Erdheim-Chester
J. Knitza, E. Kampylafka, J. Wacker, G. Schett, B. Manger
Zeitschrift für Rheumatologie.2019; 78(1): 66. CrossRef - New causes of hypophysitis
Kevin C.J. Yuen, Vera Popovic, Peter J. Trainer
Best Practice & Research Clinical Endocrinology & Metabolism.2019; 33(2): 101276. CrossRef - Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement
Myunghee Kang, Na Rae Kim, Dong Hae Chung, Jae Yeon Seok, Dong Young Kim
Journal of Pathology and Translational Medicine.2019; 53(4): 261. CrossRef - Interferon-α
Reactions Weekly.2018; 1709(1): 128. CrossRef
- Pathologic characteristics of histiocytic and dendritic cell neoplasms
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