Outcomes of adults and children with primary mediastinal B‐cell lymphoma treated with dose‐adjusted <scp>EPOCH</scp>‐R

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

doi:10.1111/bjh.14951

Cited by 107 publications

(116 citation statements)

References 28 publications

(31 reference statements)

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“…A retrospective study in 2017 supported these results when it reported on 156 adults and children with PMBCL who received DA‐EPOCH‐R. The estimated 3‐year EFS was 85·9% (95% CI 80·3–91·5%) and OS 95·4% (95% CI 91·8–99·0%); there were no differences in outcomes between adults and children (Giulino‐Roth et al , ). In this study, 14·9% patients received RT.…”

Section: Current First‐line Treatmentmentioning

confidence: 87%

Biology and therapy of primary mediastinal B‐cell lymphoma: current status and future directions

et al. 2019

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Summary Primary mediastinal B‐cell lymphoma ( PMBCL ) is a distinct disease closely related to classical nodular sclerosing Hodgkin lymphoma. Conventional diagnostic paradigms utilising clinical, morphological and immunophenotypical features can be challenging due to overlapping features with other B‐cell lymphomas. Reliable diagnostic and prognostic biomarkers that are applicable to the conventional diagnostic laboratory are largely lacking. Nuclear factor kappa B ( NF ‐κB) and Janus kinase/signal transducers and activators of transcription ( JAK ‐ STAT ) signalling pathways are characteristically dysregulated in PMBCL and implicated in several aspects of disease pathogenesis, and the latter pathway in host immune evasion. The tumour microenvironment is manipulated by PMBCL tumours to avoid T‐cell mediated destruction via strategies that include loss of tumour cell antigenicity, T‐cell exhaustion and activation of suppressive T‐regulatory cells. R‐ CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and DA ‐ EPOCH ‐R (dose‐adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) are the most common first‐line immunochemotherapy regimens. End of treatment positron emission tomography scans are the recommended imaging modality and are being evaluated to stratify patients for radiotherapy. Relapsed/refractory disease has a relatively poor outcome despite salvage immunochemotherapy and subsequent autologous stem cell transplantation. Novel therapies are therefore being developed for treatment‐resistant disease, targeting aberrant cellular signalling and immune evasion.

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Section: Current First‐line Treatmentmentioning

confidence: 87%

Biology and therapy of primary mediastinal B‐cell lymphoma: current status and future directions

et al. 2019

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“…These results have appeared also in other studies. 12,13,[26][27][28][29] There was one, however, where the outcome in patients treated with R-CHOP was poor. 22 Surprisingly, we observed a trend toward an increased relapse rate after an initial DA-EPOCH-R treatment compared to R-CHOP.…”

Section: Response Assessment and Survival Analysismentioning

confidence: 99%

Outcomes of treatment with dose‐adjusted EPOCH‐R or R‐CHOP in primary mediastinal large B‐cell lymphoma

Malenda

Kołkowska‐Leśniak

Puła

et al. 2019

European J of Haematology

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Background The standard first‐line treatment for primary mediastinal B‐cell lymphoma (PMBCL) patients is rituximab‐based immunochemotherapy; however, this is not due to the result of randomized clinical trials. Aims We retrospectively investigated 53 PMBCL patient outcomes treated either with R‐CHOP‐21 or DA‐EPOCH‐R‐28. The primary endpoint was overall survival (OS). Secondary endpoints were complete remission (CR), overall response rate (ORR), progression‐free survival (PFS), and treatment‐related complications. Results Treatment with R‐CHOP‐21 resulted in a 92.0% ORR (60% CR), while DA‐EPOCH‐R yielded a 92.6% ORR (70.4% CR). There were no differences in the occurrence of grade 3‐4 hematological adverse events, but grade 1‐2 cardiologic complications (P = .003) were observed more frequently in the DA‐EPOCH‐R arm. Median PFS and OS were not achieved. The differences in estimated 12‐month PFS in R‐CHOP and DA‐EPOCH‐R group (87% vs 73.9%) and OS (100% vs 92%) were insignificant. Patients treated with R‐CHOP‐21 and autologous hematopoietic stem cell transplantation (auto‐HSCT) had an improved OS (P = .03) but not PFS (P = .43) compared to those treated solely with R‐CHOP‐21. No differences in PFS or OS were observed between patients treated with R‐CHOP‐21/auto‐HSCT and DA‐EPOCH‐R. Conclusion The results of this study suggest that R‐CHOP‐21 may be an alternative to DA‐EPOCH‐R treatment for PMBCL patients.

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“…PMBCL patients had worse outcomes compared to other DLBCL patients [33,34]. Recently, the efficacy of rituximab in pediatric PMBCL patients has been reported [35,36]. We included five PMBCL patients, of which two showed recurrence.…”

Section: Discussionmentioning

confidence: 99%

Nitrosourea, etoposide and cyclophosphamide followed by autologous stem cell transplantation for pediatric lymphoma patients

Choi

Kang

et al. 2020

Int J Hematol

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Treatment outcomes in pediatric lymphoma have improved substantially over the past 2 decades; however, the prognosis for patients with high risk or relapsed disease remains poor. We evaluated outcomes of high-dose chemotherapy (HDC) and autologous stem cell transplantation (auto-SCT) in 56 pediatric lymphoma patients. Patients received nitrosourea (51 BCNU; 5 ACNU), etoposide, and cyclophosphamide (BEC; AEC). Median age at HDC/auto-SCT was 12 years (range 2-17 years). Forty-four patients underwent HDC/auto-SCT because they did not achieve complete remission after induction chemotherapy. Eight patients showed relapse and four NK/T-cell lymphoma patients also underwent HDC/auto-SCT. BCNU pneumonitis was diagnosed in nine (16.0%) patients. Eight (14.3%) relapsed after HDC/auto-SCT. Treatment-related mortality occurred in three cases. Five-year event-free survival and overall survival rates were 74.8% [72.7% non-Hodgkin's lymphoma (NHL); 83.3% Hodgkin's disease (HD); 72.7%] and 83.6% (81.6% NHL; 91.7% HD), respectively. HDC/auto-SCT with BEC or AEC regimen for pediatric high-risk lymphoma patients showed feasible outcomes. However, treatment modifications are warranted to reduce relapse and toxicity.

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

Cited by 107 publications

References 28 publications

Biology and therapy of primary mediastinal B‐cell lymphoma: current status and future directions

Biology and therapy of primary mediastinal B‐cell lymphoma: current status and future directions

Outcomes of treatment with dose‐adjusted EPOCH‐R or R‐CHOP in primary mediastinal large B‐cell lymphoma

Nitrosourea, etoposide and cyclophosphamide followed by autologous stem cell transplantation for pediatric lymphoma patients

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