Outcomes of Burkitt lymphoma with central nervous system involvement: Evidence from a large multicenter cohort study

Adam S. Zayac, Andrew M. Evens, Alexey Danilov, Stephen D. Smith, Deepa Jagadeesh, Lori A. Leslie, Catherine Wei, Seo Hyun Kim, Seema Naik, Suchitra Sundaram, Nishitha Reddy, Umar Farooq, Vaishalee P. Kenkre, Narendranath Epperla, Kristie A. Blum, Nadia Khan, Daulath Singh, Juan P. Alderuccio, Amandeep Godara, Maryam Sarraf YazdyCatherine Diefenbach, Emma Rabinovich, Gaurav Varma, Reem Karmali, Yusra Shao, Asaad Trabolsi, Madelyn Burkart, Peter Martin, Sarah Stettner, Ayushi Chauhan, Yun Kyong Choi, Allandria Straker-Edwards, Andreas Klein, Michael C. Churnetski, Kirsten M. Boughan, Stephanie Berg, Bradley M. Haverkos, Victor M. Orellana-Noia, Christopher D'Angelo, David A. Bond, Seth M. Maliske, Ryan Vaca, Gabriella Magarelli, Amy Sperling, Max J. Gordon, Kevin A. David, Malvi Savani, Paolo Caimi, Manali Kamdar, Matthew A. Lunning, Neil Palmisiano, Parameswaran Venugopal, Craig A. Portell, Veronika Bachanova, Tycel Phillips, Izidore S. Lossos, Adam J. Olszewski

Research output: Contribution to journalArticlepeer-review

15 Scopus citations


Central nervous system (CNS) involvement in Burkitt lymphoma poses a major therapeutic challenge, and the relative ability of contemporary regimens to treat CNS involvement remains uncertain. We describe the prognostic significance of CNS involvement and the incidence of CNS recurrence/progression after contemporary immunochemotherapy using real-world clinicopathological data from adults with Burkitt lymphoma diagnosed between 2009 and 2018 in 30 institutions in the USA. We examined associations between baseline CNS involvement, patients' characteristics, complete response rates, and survival. We also examined risk factors for CNS recurrence. Of 641 patients (aged 18 to 88 years), 120 (19%) had CNS involvement. CNS involvement was independently associated with human immunodeficiency virus infection, poor performance status, involvement of ≥2 extranodal sites, and bone marrow involvement. Selection of the first-line treatment regimen was unaffected by CNS involvement (P=0.93). Patients with CNS disease had significantly lower rates of complete response (59% vs. 77% for patients with and without CNS involvement, respectively; P<0.001), worse 3-year progression-free survival (adjusted hazard ratio [aHR]=1.53, 95% confidence interval [95% CI]: 1.14-2.06; P=0.004) and overall survival (aHR=1.62, 95% CI: 1.18-2.22; P=0.003). The 3-year cumulative incidence of CNS recurrence was 6% (95% CI: 4-8%) and was significantly lower among patients receiving other regimens (CODOX-M/IVAC, 4%, or hyperCVAD/MA, 3%) compared with DA-EPOCH-R (13%; adjusted sub-distribution HR=4.38, 95% CI:, 2.16-8.87; P<0.001). Baseline CNS involvement in Burkitt lymphoma is relatively common and portends inferior prognosis independently of the first-line treatment regimen selected. In real-world practice, regimens including intravenous systemic agents with pronounced CNS penetrance were associated with a lower risk of CNS recurrence. This finding may be influenced by observed suboptimal adherence to the strict CNS staging and intrathecal therapy procedures incorporated in the DA-EPOCH-R regimen.

Original languageEnglish (US)
Pages (from-to)1932-1942
Number of pages11
Issue number7
StatePublished - Jul 2021

ASJC Scopus subject areas

  • Hematology


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