Epstein-barr virus-infected cells in IgG4-related lymphadenopathy with comparison with extranodal IgG4-related disease

Mai Takeuchi, Yasuharu Sato, Hiroshi Yasui, Hiroaki Ozawa, Kyotaro Ohno, Katsuyoshi Takata, Yuka Gion, Yorihisa Orita, Tomoyasu Tachibana, Tomoo Itoh, Naoko Asano, Shigeo Nakamura, Steven H. Swerdlow, Tadashi Yoshino

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15 Citations (Scopus)

Abstract

IgG4-related lymphadenopathy with increased numbers of Epstein-Barr virus (EBV)-infected cells has been reported but not fully described. We analyzed 31 cases of IgG4-related lymphadenopathy and 24 cases of extranodal IgG4-related diseases for their possible relationship with EBV. Other types of reactive lymph nodes (22) and angioimmunoblastic T-cell lymphoma (AITL) (10) were also studied for comparison. EBV-encoded RNA (EBER) in situ hybridization revealed EBER + cells in 18 of 31 cases (58%) of IgG4-related lymphadenopathy. Increased EBER+ cells were found in only 4 of 22 (18.1%) non-IgG4-related reactive lymphoid hyperplasia in patients of a similar age (P=0.002) and in only 5 of 24 (21%) extranodal IgG4-related biopsies (P=0.006). Interestingly, all patients with EBER+ progressively transformed germinal center-type IgG4-related lymphadenopathy had systemic lymphadenopathy and/or extranodal involvement. AITL also is associated with EBV, and IgG4-related lymphadenopathy sometimes mimics the morphology of AITL; however, the number of IgG4+ cells in AITL was significantly less than that in IgG4-related lymphadenopathy (P<0.001). Increased numbers of regulatory T cells are seen in IgG4-related disease; however, there was not a significant difference between the EBER+ and EBER- cases. In conclusion, the presence of increased numbers of EBV-infected cells in IgG4-related lymphadenopathy, compared with other reactive lymphadenopathy or extranodal IgG4-related disease, suggests that there may be a relationship at least between nodal IgG4-related disease and EBV. It is important to avoid overdiagnosing these cases as malignant lymphomas or EBV-related lymphoproliferative disorders.

Original languageEnglish
Pages (from-to)946-955
Number of pages10
JournalAmerican Journal of Surgical Pathology
Volume38
Issue number7
DOIs
Publication statusPublished - Jul 2014

Keywords

  • Epstein-Barr virus
  • IgG4-related disease
  • IgG4-related lymphadenopathy

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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    Takeuchi, M., Sato, Y., Yasui, H., Ozawa, H., Ohno, K., Takata, K., Gion, Y., Orita, Y., Tachibana, T., Itoh, T., Asano, N., Nakamura, S., Swerdlow, S. H., & Yoshino, T. (2014). Epstein-barr virus-infected cells in IgG4-related lymphadenopathy with comparison with extranodal IgG4-related disease. American Journal of Surgical Pathology, 38(7), 946-955. https://doi.org/10.1097/PAS.0000000000000206