title: Clinicopathological Analysis of Ocular Adnexal Extranodal Marginal Zone B-Cell Lymphoma with IgG4-Positive Cells.
abstract: This study aims to analyze clinical and pathological characteristics of ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL) accompanying IgG4-positive cells. Fifty patients with a diagnosis of primary non-conjunctival ocular adnexal EMZL were enrolled in this study. The number of IgG4-positive cells and the ratio of IgG/IgG4 were evaluated by immunohistochemistry in the biopsy specimens. The patients were divided into two groups based on the absolute number and the ratio of IgG4-positive cells (IgG4-posivite vs IgG4-negative groups). The demographic data, clinical staging at diagnosis, histopathological characteristics, and response to initial treatment were comparatively analyzed between the 2 groups. Five (10%) of 50 patients were defined as IgG4-positive group, and all the cases showed characteristic histological features such as extensive plasma cell infiltration and dense fibrosis. Most of these patients (4 of 5 patients) had lymphoma of the lacrimal gland. The patients from the IgG4-positive group showed a lower response rate to initial treatment (87.5 vs 33%, p = 0.03) than IgG4-negative group with a median follow-up period of 38 months. A part of the ocular adnexal EMZLs were accompanied with IgG4-positive cells. Significantly, most IgG4-positive ocular adnexal EMZLs occurred in the lacrimal gland, and can be related with a more frequent treatment failure.
authors.affiliation: Department of Ophthalmology, Hallym University Sacred Heart Hospital, Anyang, Korea.
authors.forename: Min Joung
authors.initials: MJ
authors.lastname: Lee
authors.affiliation: Department of Ophthalmology, Seoul National University Bundang Hospital, Seungnam, Korea.
authors.forename: Namju
authors.initials: N
authors.lastname: Kim
authors.affiliation: Department of Pathology, Seoul National University Bundang Hospital, Seungnam, Korea.
authors.forename: Ji-Young
authors.initials: JY
authors.lastname: Choe
authors.affiliation: Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Sang In
authors.initials: SI
authors.lastname: Khwarg
authors.affiliation: Department of Pathology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Yoon Kyung
authors.initials: YK
authors.lastname: Jeon
authors.affiliation: Department of Ophthalmology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Ho-Kyung
authors.initials: HK
authors.lastname: Choung
authors.affiliation: Department of Pathology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Ji Eun
authors.initials: JE
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D005134:Eye Neoplasms / Q000276:immunology / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000378:metabolism*; D007150:Immunohistochemistry; D018442:Lymphoma, B-Cell, Marginal Zone / Q000276:immunology / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G