Doença de Erdheim-Chester: relato de dois casos / Erdheim-Chester disease: a two-case report
Radiol. bras
;
42(4): 267-269, jul.-ago. 2009. ilus
Article
in English, Portuguese
| LILACS
| ID: lil-524407
RESUMO
A doença de Erdheim-Chester é uma rara histiocitose de células não-Langerhans, de etiologia desconhecida, que apresenta manifestações sistêmicas, atingindo ossos, sistema nervoso central, olhos, pulmões, mediastino, rins e retroperitônio. Relatamos dois casos que cursaram com a apresentação típica da doença. Os achados radiológicos foram concordantes com a literatura e orientaram a suspeita diagnóstica, confirmada pelo exame imuno-histoquímico.
ABSTRACT
Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, affecting multiple organ system, involving bones, central nervous system, eyes, lungs, mediastinum, kidneys and retroperitoneum. The authors report two cases that progressed with the typical presentation of the disease. Radiological findings were in agreement with literature and guided the diagnosis, confirmed by immunohistochemistry.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Histiocytosis, Non-Langerhans-Cell
/
Erdheim-Chester Disease
Limits:
Adult
/
Female
/
Humans
Language:
English
/
Portuguese
Journal:
Radiol. bras
Journal subject:
Diagnostic Imaging
/
Radiology
Year:
2009
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Clínica Imagem/BR
/
Hospital Bruno Born/BR
/
Santa Casa de Misericórdia de Porto Alegre/BR
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