title: Erdheim-Chester disease with prominent pericardial effusion: cytologic findings and review of the literature.
abstract: Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin with distinct clinicopathologic and radiographic features. Reports detailing the cytology of ECD are rare. We describe a case of ECD with pericardial effusion. Cytologic examination revealed a hypercellular specimen composed of clusters and singly dispersed foamy macrophages with round nuclei and inconspicuous nucleoli, admixed with lymphocytes, eosinophils, and Touton-type multinucleated giant cells. Immunostains for CD68 were strongly positive in the foamy macrophages while S100 and CD1a were negative. The presence of foamy histiocytes, multinucleated giant cells, lymphocytes and eosinophils are also features of other systemic histiocytic disorders, including Langerhans cell histiocytosis (LCH), Rosai-Dorfman disease (RDD) and sarcoidosis. To the best of out knowledge, this is the first report describing the cytological features of ECD in a pericardial effusion.
authors: Department of Pathology, University of Maryland Medical Center, Baltimore, Maryland.
authors: Borislav A
authors: BA
authors: Alexiev
authors: Paul N
authors: PN
authors: Staats
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000235:genetics / Q000378:metabolism; D018949:Antigens, CD1 / Q000235:genetics / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000235:genetics / Q000378:metabolism; D031249:Erdheim-Chester Disease / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D006801:Humans; D010490:Pericardial Effusion / Q000378:metabolism / Q000473:pathology*; D009418:S100 Proteins / Q000235:genetics / Q000378:metabolism; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D018949:Antigens, CD1; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C419393:CD1a antigen; C067980:CD68 antigen, human; D000097382:CD68 Molecule
keywords: Erdheim-Chester disease; cytology