title: Lymphomatoid granulomatosis--a single institute experience: pathologic findings and clinical correlations.
abstract: Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder. It is hypothesized that these patients have dysregulated immune surveillance of EBV. We reviewed the biopsies of 55 patients with LYG who were referred for a prospective trial at the National Cancer Institute (1995 to 2010) and evaluated the histologic, immunohistochemical, in situ hybridization, and molecular findings of these biopsies in conjunction with clinical information. Grading of the lesions was based on morphologic features and the number of EBV-positive B cells. The median age was 46 years (M:F 2.2:1). Clinically, all patients had lung involvement (100%), with the next most common site being the central nervous system (38%). No patient had nodal or bone marrow disease. All patients had past EBV exposure by serology but with a low median EBV viral load. We reviewed 122 biopsies; the most common site was lung (73%), followed by skin/subcutaneous tissue (17%); other sites included kidney, nasal cavity, gastrointestinal tract, conjunctiva, liver, and adrenal gland. Histologically, the lesions showed angiocentricity, were rich in T cells, had large atypical B cells, and were positive for EBV. Grading was performed predominantly on the lung biopsy at diagnosis; they were distributed as follows: LYG grade 1 (30%), grade 2 (22%), and grade 3 (48%). Necrosis was seen in all grades, with a greater degree in high-grade lesions. Immunoglobulin gene rearrangement studies were performed, and a higher percentage of clonal rearrangements were seen in LYG grade 2 (50%) and grade 3 (69%) as compared with grade 1 (8%). LYG is a distinct entity that can usually be differentiated from other EBV-associated B-cell lymphoproliferative disorders on the basis of the combination of clinical presentation, histology, and EBV studies. Grading of these lesions is important because it dictates the treatment choice.
authors.affiliation: *Laboratory of Pathology ‡Metabolism Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD †Department of Pathology and Laboratory Medicine, City of Hope Medical Center, Duarte, CA §Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FL.
authors.forename: Joo Y
authors.initials: JY
authors.lastname: Song
authors.forename: Stefania
authors.initials: S
authors.lastname: Pittaluga
authors.forename: Kieron
authors.initials: K
authors.lastname: Dunleavy
authors.forename: Nicole
authors.initials: N
authors.lastname: Grant
authors.forename: Therese
authors.initials: T
authors.lastname: White
authors.forename: Liuyan
authors.initials: L
authors.lastname: Jiang
authors.forename: Theresa
authors.initials: T
authors.lastname: Davies-Hill
authors.forename: Mark
authors.initials: M
authors.lastname: Raffeld
authors.forename: Wyndham H
authors.initials: WH
authors.lastname: Wilson
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
mesh_terms: D000328:Adult; D000368:Aged; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008230:Lymphomatoid Granulomatosis / Q000235:genetics / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D055815:Young Adult
chemical_list: D007143:Immunoglobulin Heavy Chains