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

• Published in: Leukemia & lymphoma 2023-04, Vol.64 (5), p.1026-1034
• Main Authors: Agrawal, Prashasti, David, Kevin A., Chen, Zhengming, Sundaram, Suchitra, Kim, Seo-Hyun, Vaca, Ryan, Lin, Yong, Singer, Samuel, Malecek, Mary-Kate, Carter, Jordan, Zayac, Adam, Kim, Myung Sun, Reddy, Nishitha, Ney, Douglas, Habib, Alma, Strouse, Christopher, Graber, Jerome, Bachanova, Veronika, Salman, Sidra, Vendiola, Jean A., Hossain, Nasheed, Tsang, Mazie, Major, Ajay, Gandhi, Maher K., Keane, Colm, Bond, David A., Folstad, Matthew, Chang, Julie, Mier-Hicks, Angel, Torka, Pallawi, Rajakumar, Priya, Venugopal, Parameswaran, Berg, Stephanie, Glantz, Michael, Goldlust, Samuel A., Matnani, Rahul, Kumar, Pallavi, Ollila, Thomas A., Cai, Johnny, Spurgeon, Stephen E., Sieg, Alex G., Cleveland, Joseph, Epperla, Narendranath, Karmali, Reem, Naik, Seema, Smith, Sonali M., Rubenstein, James L., Kahl, Brad S., Chadburn, Amy, Evens, Andrew M., Martin, Peter
• Format: Article
• Language: English
• Subjects: Epstein-Barr virus (EBV); post-transplant Lymphoproliferative Disorders (PTLD); primary CNS lymphoma (PCNSL)
• ISSN: 1042-8194; 1029-2403
• DOI: 10.1080/10428194.2023.2191152

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.