title: Primary IgG4-related lymphadenopathy with prominent granulomatous inflammation and reactivation of Epstein-Barr virus.
abstract: We report a unique case of primary IgG4-related lymphadenopathy showing prominent granulomatous inflammation and Epstein-Barr virus (EBV) reactivation. Involved lymph nodes showed an expanded interfollicular zone with prominent granulomatous inflammation, including a predominance of epithelioid macrophages and occasional Langhans multinucleated giant cells. Bundles of spindle cells were also observed. Intermingled with the granulomatous inflammation were numerous mature plasma cells, eosinophils, and neutrophils. The percentage of IgG4+/IgG+ plasma cells was markedly elevated (70%), along with raised serum IgG4 levels. The plasma cells did not show immunoglobulin light-chain restriction. EBV-positive lymphocytes were scattered throughout the paracortical areas. Corticosteroid treatment was very effective. IgG4-related lymphadenopathy has a broad histological spectrum and might be misdiagnosed due to other conditions which morphologically closely resemble it. The correct diagnosis is important in view of the remarkable response to steroid therapy.
authors.affiliation: Department of Pathology, Aichi Medical University Hospital, Nagakute, Japan. emikot@aichi-med-u.ac.jp
authors.forename: Emiko
authors.initials: E
authors.lastname: Takahashi
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.forename: Mizuki
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Atsuko
authors.initials: A
authors.lastname: Kitamura
authors.forename: Toyoharu
authors.initials: T
authors.lastname: Yokoi
authors.forename: Kazuo
authors.initials: K
authors.lastname: Hara
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000276:immunology / Q000473:pathology* / Q000821:virology*; D005260:Female; D006099:Granuloma / Q000276:immunology / Q000473:pathology / Q000821:virology; D004854:Herpesvirus 4, Human / Q000502:physiology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007249:Inflammation / Q000276:immunology / Q000473:pathology / Q000821:virology; D008206:Lymphatic Diseases / Q000276:immunology / Q000473:pathology* / Q000821:virology*; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D014775:Virus Activation
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D011239:Prednisolone