Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement.
10.4046/trd.2009.67.4.345
- Author:
Ji Won PARK
1
;
Chae Uk CHUNG
;
Ji Young SHIN
;
Sun Young JUNG
;
Su Jin YOO
;
Jeong Eun LEE
;
Sung Soo JUNG
;
Ju Ock KIM
;
Sun Young KIM
;
Hee Sun PARK
Author Information
1. Department of Internal Medicine, College of Medicine, Chungnam National University, Daejeon, Korea. liansalt@hanmail.net
- Publication Type:Case Report
- Keywords:
Erdheim-Chester disease;
Glomerulonephritis;
Aplastic anemia;
Hepatitis;
Lung involvement
- MeSH:
Adult;
Anemia, Aplastic;
Biopsy;
Diagnosis, Differential;
Edema;
Erdheim-Chester Disease;
Glomerulonephritis;
Hepatitis;
Histiocytosis;
Humans;
Lost to Follow-Up;
Lung;
Rare Diseases;
S100 Proteins;
Transaminases
- From:Tuberculosis and Respiratory Diseases
2009;67(4):345-350
- CountryRepublic of Korea
- Language:English
-
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.
