Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement / 결핵및호흡기질환
Tuberculosis and Respiratory Diseases
;
: 345-350, 2009.
Artigo
em Inglês
| WPRIM
| ID: wpr-190769
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Biópsia
/
Proteínas S100
/
Histiocitose
/
Doença de Erdheim-Chester
/
Doenças Raras
/
Diagnóstico Diferencial
/
Edema
/
Perda de Seguimento
/
Glomerulonefrite
/
Transaminases
Tipo de estudo:
Estudo diagnóstico
Limite:
Adulto
/
Humanos
Idioma:
Inglês
Revista:
Tuberculosis and Respiratory Diseases
Ano de publicação:
2009
Tipo de documento:
Artigo
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