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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 

Original publication

DOI

10.1111/bjh.14951

Type

Journal article

Journal

Br J Haematol

Publication Date

12/2017

Volume

179

Pages

739 - 747

Keywords

DA-EPOCH-R, non-Hodgkin lymphoma, paediatric oncology, primary mediastinal B-cell lymphoma, Adolescent, Adult, Age Factors, Aged, Antineoplastic Combined Chemotherapy Protocols, Child, Cyclophosphamide, Doxorubicin, Drug Administration Schedule, Etoposide, Female, Humans, Kaplan-Meier Estimate, Lymphoma, Large B-Cell, Diffuse, Male, Mediastinal Neoplasms, Middle Aged, Neoplasm Staging, Positron-Emission Tomography, Prednisone, Prognosis, Radiotherapy, Adjuvant, Retrospective Studies, Rituximab, Thrombosis, Treatment Outcome, Vincristine, Young Adult