EBV-positive PCNSL in older patients: incidence, characteristics, tumor pathology, and outcomes across a large multicenter cohort

Prashasti Agrawal, Kevin A. David, Zhengming Chen, Suchitra Sundaram, Seo Hyun Kim, Ryan Vaca, Yong Lin, Samuel Singer, Mary Kate Malecek, Jordan Carter, Adam Zayac, Myung Sun Kim, Nishitha Reddy, Douglas Ney, Alma Habib, Christopher Strouse, Jerome Graber, Veronika Bachanova, Sidra Salman, Jean A. VendiolaNasheed Hossain, Mazie Tsang, Ajay Major, Maher K. Gandhi, Colm Keane, David A. Bond, Matthew Folstad, Julie Chang, Angel Mier-Hicks, Pallawi Torka, Priya Rajakumar, Parameswaran Venugopal, Stephanie Berg, Michael Glantz, Samuel A. Goldlust, Rahul Matnani, Pallavi Kumar, Thomas A. Ollila, Johnny Cai, Stephen E. Spurgeon, Alex G. Sieg, Joseph Cleveland, Narendranath Epperla, Reem Karmali, Seema Naik, Sonali M. Smith, James L. Rubenstein, Brad S. Kahl, Amy Chadburn, Andrew M. Evens, Peter Martin

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

The objective of this multicenter retrospective study was to examine the incidence, patient characteristics, pathology, and outcomes associated with Epstein-Barr virus (EBV)-related CNS lymphoma (CNSL) in older patients. Among 309 CNSL patients aged ≥60, 11.7% had EBV + tumors of which 72.2% were solid organ transplant (SOT)-related post-transplant lymphoproliferative disorders (PTLD). Younger age, SOT or autoimmune disease, and immunosuppressive treatment correlated highly with EBV-positivity. EBV + tumors were associated with absent C-MYC and BCL6 expression. EBV + PTLD was more likely to be associated with the absence of CD5 expression. EBV + non-PTLD had better median OS (not reached) compared to EBV + PTLD (10.8 months) and EBV-negative patients (43 months). Multivariable Cox regression analysis showed that age, performance status, and PTLD were negative predictors of OS. EBV status and immunosuppressive treatment were not correlated with OS. Our findings merit further investigation of EBV + PCNSL tumors and EBV-directed therapies.

Original languageEnglish
Pages (from-to)1026-1034
Number of pages9
JournalLeukemia and Lymphoma
Volume64
Issue number5
DOIs
StatePublished - 2023

Keywords

  • Epstein-Barr virus (EBV)
  • post-transplant Lymphoproliferative Disorders (PTLD)
  • primary CNS lymphoma (PCNSL)

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