title: Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes.
abstract: Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4(+) progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4(+) plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4(+) progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all responded well. We suggest that IgG4(+) progressively transformed germinal centers should be included in the IgG4-related disease spectrum.
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. satou-y@cc.okayama-u.ac.jp
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.forename: Dai
authors.initials: D
authors.lastname: Inoue
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Hideki
authors.initials: H
authors.lastname: Asaoku
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Maeda
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Morito
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Okumura
authors.forename: Shin
authors.initials: S
authors.lastname: Ishizawa
authors.forename: Shoko
authors.initials: S
authors.lastname: Matsui
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Miyazono
authors.forename: Tamotsu
authors.initials: T
authors.lastname: Takeuchi
authors.forename: Naoto
authors.initials: N
authors.lastname: Kuroda
authors.forename: Yorihisa
authors.initials: Y
authors.lastname: Orita
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Takagawa
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D018858:Germinal Center / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G; D007150:Immunohistochemistry; D008206:Lymphatic Diseases / Q000473:pathology*; D008297:Male; D008875:Middle Aged
chemical_list: D007074:Immunoglobulin G