Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R

Lisa Giulino-Roth, Tara O'Donohue, Zhengming Chen, Nancy L. Bartlett, Ann LaCasce, William Martin-Doyle, Matthew J. Barth, Kimberly Davies, Kristie A. Blum, Beth Christian, Carla Casulo, Sonali M. Smith, James Godfrey, Amanda Termuhlen, Matthew J. Oberley, Sarah Alexander, Sheila Weitzman, Burton Appel, Benjamin Mizukawa, Jakub SvobodaZeinab Afify, Melinda Pauly, Hema Dave, Rebecca Gardner, Deborah M. Stephens, William A. Zeitler, Christopher Forlenza, Jennifer Levine, Michael E. Williams, Jody L. Sima, Catherine M. Bollard, John P. Leonard

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99 Scopus citations


Treatment with dose-adjusted EPOCH (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone) chemotherapy and rituximab (DA-EPOCH-R) has become the standard of care for primary mediastinal B-cell lymphoma (PMBCL) at many institutions despite limited data in the multi-centre setting. We report a large, multi-centre retrospective analysis of children and adults with PMBCL treated with DA-EPOCH-R to characterize outcomes and evaluate prognostic factors. We assessed 156 patients with PMBCL treated with DA-EPOCH-R across 24 academic centres, including 38 children and 118 adults. All patients received at least one cycle of DA-EPOCH-R. Radiation therapy was administered in 14·9% of patients. With median follow-up of 22·6 months, the estimated 3-year event-free survival (EFS) was 85·9% [95% confidence interval (CI) 80·3–91·5] and overall survival was 95·4% (95% CI 91·8–99·0). Outcomes were not statistically different between paediatric and adult patients. Thrombotic complications were reported in 28·2% of patients and were more common in paediatric patients (45·9% vs. 22·9%, P = 0·011). Seventy-five per cent of patients had a negative fluorodeoxyglucose positron emission tomography (FDG-PET) scan at the completion of DA-EPOCH-R, defined as Deauville score 1–3. Negative FDG-PET at end-of-therapy was associated with improved EFS (95·4% vs. 54·9%, P < 0·001). Our data support the use of DA-EPOCH-R for the treatment of PMBCL in children and adults. Patients with a positive end-of-therapy FDG-PET scan have an inferior outcome.

Original languageEnglish
Pages (from-to)739-747
Number of pages9
JournalBritish Journal of Haematology
Issue number5
StatePublished - Dec 2017


  • non-Hodgkin lymphoma
  • paediatric oncology
  • primary mediastinal B-cell lymphoma


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