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Vol.67 No.4
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Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement

Tuberculosis & Respiratory Diseases / Tuberculosis & Respiratory Diseases,
2009, v.67 no.4, pp.345-350
Ji Won Park (Chungnam National University)
Chae Uk Chung (Chungnam National University)
Ji Young Shin (Chungnam National University)
Sun Young Jung (Chungnam National University)
Su Jin Yoo (Chungnam National University)



Sun Young Kim (Chungnam National University)

Ji, W. P. , Chae, U. C. , Ji, Y. S. , Sun, Y. J. , Su, J. Y. , Sun, Y. K. , & (2009). Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement. Tuberculosis & Respiratory Diseases, 67(4), 345-350.
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Abstract

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

keywords
Erdheim-Chester disease, Glomerulonephritis, Aplastic anemia, Hepatitis, Lung involvement

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