Expanding the Spectrum of EBV-positive Marginal Zone Lymphomas: A Lesion Associated With Diverse Immunodeficiency Settings

Shunyou Gong, Genevieve M. Crane, Chad M. McCall, Wenbin Xiao, Karthik A. Ganapathi, Nathan Cuka, Theresa Davies-Hill, Liqiang Xi, Mark Raffeld, Stefania Pittaluga, Amy S. Duffield, Elaine S. Jaffe*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Traditionally low-grade B-cell lymphomas have been excluded from the category of monomorphic posttransplant lymphoproliferative disorders. However, recent reports identified Epstein-Barr virus-positive (EBV+) extranodal marginal zone lymphomas (MZL), almost exclusively seen in the posttransplant setting. Some reported cases responded to reduced immunosuppression, suggesting that they should be considered as a form of posttransplant lymphoproliferative disorders. We identified 10 cases of EBV+ MZL, 9 in extranodal sites and 1 presenting in lymph node. Two cases arose following solid organ transplantation, but other settings included iatrogenic immunosuppression for rheumatoid arthritis (2); prior chemotherapy (2); congenital immune deficiency (1); and increased age (3), as the only potential cause of immune dysfunction. There were 4 males and 6 females; age range 18 to 86. The atypical plasmacytoid and/or monocytoid B cells were positive for EBV in all cases, with either latency I or II in all cases tested. Monotypic light chain expression was shown in all with 6 cases positive for IgG, and 2 for IgM, undetermined in 2. Clonal immunoglobulin gene rearrangement was positive in all cases with successful amplification. MYD88 L265P was wild type in the 6 cases tested. We show that EBV+ MZLs can arise in a variety of clinical settings, and are most often extranodal. Treatment varied, but most patients had clinically indolent disease with response to reduction of immune suppression, or immunochemotherapy.

Original languageEnglish (US)
Pages (from-to)1306-1316
Number of pages11
JournalAmerican Journal of Surgical Pathology
Issue number10
StateAccepted/In press - Jun 27 2018


  • Epstein-Barr virus
  • immunodeficiency
  • immunosenescence
  • immunosuppression
  • MALT lymphoma
  • marginal zone lymphoma
  • posttransplant lymphoproliferative disease

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine


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