Lymphomatoid granulomatosis-a single institute experience: Pathologic findings and clinical correlations

Joo Y. Song, Stefania Pittaluga, Kieron Dunleavy, Nicole Grant, Therese White, Liuyan Jiang, Theresa Davies-Hill, Mark Raffeld, Wyndham H. Wilson, Elaine S. Jaffe

Research output: Contribution to journalArticle

47 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)141-156
Number of pages16
JournalAmerican Journal of Surgical Pathology
Volume39
Issue number2
DOIs
StatePublished - Feb 2 2015
Externally publishedYes

Keywords

  • Epstein-Barr virus
  • immunodeficiency
  • immunosurveillance
  • lung
  • lymphomatoid granulomatosis
  • lymphoproliferative disorder

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

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    Song, J. Y., Pittaluga, S., Dunleavy, K., Grant, N., White, T., Jiang, L., Davies-Hill, T., Raffeld, M., Wilson, W. H., & Jaffe, E. S. (2015). Lymphomatoid granulomatosis-a single institute experience: Pathologic findings and clinical correlations. American Journal of Surgical Pathology, 39(2), 141-156. https://doi.org/10.1097/PAS.0000000000000328