title: Immunopathological analysis of Erdheim-Chester disease with massive ascites.
abstract: We treated a 77-year-old woman with pleural and pericardial effusion and ascites. Initially, collagen vascular disease was suspected due to the presence of anti-centromere antibodies and suspected complication of pulmonary arterial hypertension. However, soft-tissue abnormalities surrounding the bilateral kidneys detected on computed tomography (CT) and symmetrical lesions of the long bones detected on bone scintigraphy made us consider a diagnosis of Erdheim-Chester disease (ECD), which is a rare form of histiocytosis. We immunochemically analyzed the cells derived from the ascites in detail and confirmed the diagnosis. Immunocytochemical analyses may therefore help to achieve a better understanding of the pathogenesis of this rare disease.
authors.affiliation: Department of Rheumatology and Applied Immunology, Faculty of Medicine, Saitama Medical University, Japan.
authors.forename: Muneo
authors.initials: M
authors.lastname: Ota
authors.forename: Mayuko
authors.initials: M
authors.lastname: Sakamoto
authors.forename: Kojiro
authors.initials: K
authors.lastname: Sato
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Yu
authors.initials: Y
authors.lastname: Funakubo Asanuma
authors.forename: Yuji
authors.initials: Y
authors.lastname: Akiyama
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Yamakawa
authors.forename: Toshihide
authors.initials: T
authors.lastname: Mimura
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001201:Ascites / Q000276:immunology / Q000473:pathology; D001842:Bone and Bones / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000378:metabolism; D007668:Kidney / Q000473:pathology; D008279:Magnetic Resonance Imaging; D011877:Radionuclide Imaging; D011956:Receptors, Cell Surface / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D000098902:CD163 Antigen
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000098902:CD163 Antigen; D019394:Ki-67 Antigen; D011956:Receptors, Cell Surface