Erdheim-Chester Disease with Emperipolesis: A Unique Case Involving the Heart / Journal of the Korean Cancer Association, 대한암학회지
Cancer Research and Treatment
;
: 553-558, 2017.
Artigo
em Inglês
| WPRIM
| ID: wpr-63848
ABSTRACT
Histiocytosis is an uncommon disease characterized by excessive accumulation of histiocytes. Here, we report a rare case of non-Langerhans-cell histiocytosis in a 51-year-old woman who presented with severe symptoms of pericardial effusion. Radiologic investigation also detected multiple bone (lower limbs, vertebrae, ribs, and ilium) lesions. Resected pericardium showed abundant mono- or multi-nucleated non-foamy histiocytes (CD68⁺/CD163⁺/S-100⁺/CD1α⁻/langerin⁻) in a fibroinflammatory background. The histiocytes demonstrated emperipolesis of lymphocytes, a hallmark feature of Rosai-Dorfman disease (RDD). However, molecular analysis revealed a BRAF V600E mutation of the proliferating histiocytes, highlighting the neoplastic features frequently observed in another non-Langerhans-cell histiocytosis known as Erdheim-Chester Disease (ECD). We consider this case to be a unique presentation of ECD harboring some RDD-like cells with emperipolesis, but not a case of RDD with a BRAF mutation concerning its clinical manifestation (involvement of the heart and bones) and neoplastic features.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Derrame Pericárdico
/
Pericárdio
/
Costelas
/
Coluna Vertebral
/
Linfócitos
/
Histiocitose
/
Histiocitose de Células não Langerhans
/
Histiocitose Sinusal
/
Doença de Erdheim-Chester
/
Extremidades
Limite:
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Cancer Research and Treatment
Ano de publicação:
2017
Tipo de documento:
Artigo
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