Expanding the Spectrum of EBV-positive Marginal Zone Lymphomas


Journal Article

© 2018 Wolters Kluwer Health, Inc. All rights reserved. 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.

Full Text

Duke Authors

Cited Authors

  • Gong, S; Crane, GM; McCall, CM; Xiao, W; Ganapathi, KA; Cuka, N; Davies-Hill, T; Xi, L; Raffeld, M; Pittaluga, S; Duffield, AS; Jaffe, ES

Published Date

  • October 1, 2018

Published In

Volume / Issue

  • 42 / 10

Start / End Page

  • 1306 - 1316

Electronic International Standard Serial Number (EISSN)

  • 1532-0979

International Standard Serial Number (ISSN)

  • 0147-5185

Digital Object Identifier (DOI)

  • 10.1097/PAS.0000000000001113

Citation Source

  • Scopus