title: A subset of Rosai-Dorfman disease exhibits features of IgG4-related disease.
abstract: In this study we investigated the distribution of IgG4+ plasma cells and regulatory T (T(REG)) cells, a major regulator of IgG4 production, in nodal and extranodal Rosai-Dorfman disease (RDD). Twenty-six specimens (15 nodal, 11 extranodal) were examined, with reactive lymph nodes and site-matched extranodal specimens as controls. Overall, 84.6% (22/26) of the specimens showed various degrees of sclerosis (7 mild, 8 moderate, and 7 severe). Nineteen cases (73.1%) exhibited more than 10 IgG4+ cells/0.060 mm(2) (photographed area at ×40), and 8 cases (30.8%) showed more than 40% of IgG+ cells being IgG4+. Only 1 control case exhibited more than 10 IgG4+ cells/0.060 mm(2) (P < .05). The number of T(REG) cells was comparable between nodal RDD and controls, whereas extranodal RDD exhibited significantly higher numbers of T(REG) cells than controls. These findings demonstrate that a subset of RDD shows features of IgG4-related disease and indicate an overlap between certain aspects of the 2 diseases.
authors.affiliation: Department of Pathology, University of Chicago Medical Center, Chicago, IL 60637, USA.
authors.forename: Xuefeng
authors.initials: X
authors.lastname: Zhang
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Hyjek
authors.forename: James
authors.initials: J
authors.lastname: Vardiman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D015618:Histiocytosis, Sinus / Q000276:immunology / Q000473:pathology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000276:immunology / Q000473:pathology*; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D012598:Sclerosis; D050378:T-Lymphocytes, Regulatory / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G