Erdheim-Chester disease with a late onset cardiovascular manifestations: A case report
نویسندگان
چکیده
Introduction: Erdheim-Chester disease (ECD) is an uncommon non Langerhans cell histiocytosis. Bilateral symmetric sclerosis of the long bones and probable multiorgan involvement are main manifestations this condition. Here, we report a case ECD showing diffuse thickening aorta’s entire wall pericardial effusion.
منابع مشابه
Erdheim-Chester Disease: A Case Report
A 42-year-old man with Erdheim-Chester disease (EC) is presented. This is the first case of this disease reported in Korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. Magnetic resonance imaging revealed that the...
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Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterised by multiorgan xanthogranulomatous infiltration of histiocytes (CD68+/CD1−). Two highly suggestive characteristics of this disease are sclerosis of the long bones and perirenal fat infiltration. The central nervous system (CNS) involvement occurs in 30% to 50% of cases and is the main predictor of a p...
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متن کاملErdheim-Chester Disease: Case Report with Aggressive Multisystem Manifestations and Review of the Literature
Erdheim-Chester disease (ECD) is an extremely rare and aggressive form of non-Langerhans cell histiocytosis. ECD usually presents with bone pain in adults aged 40-60. Its etiology is unknown but it is thought to be either a reactive or neoplastic disorder. Recently, mutation of the proto-oncogene BRAF (BRAFV600E) has been found in more than 50% of cases. The multisystemic form of ECD is associa...
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ژورنال
عنوان ژورنال: Journal of clinical images and medical case reports
سال: 2023
ISSN: ['2766-7820']
DOI: https://doi.org/10.52768/2766-7820/2315