HEALTH PROFESSIONAL · SOURCE READING
Chemotherapy and rituximab
Source: Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 17, 2025 · Captured 2026-09-09.
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Pediatric and adolescent patients with stage III primary mediastinal large B-cell lymphoma fared significantly worse on the FAB/LMB-96 (NCT00002757) study, with a 5-year EFS rate of 66%, compared with 85% for adolescents with nonmediastinal diffuse large B-cell lymphoma.[92][Level of evidence B4] Similarly, in the NHL-BFM-95 trial, patients with primary mediastinal B-cell lymphoma had an EFS rate of 50% at 3 years.[21] However, a study of young adults treated with DA-EPOCH-R showed excellent disease-free survival rates.[93]
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Evidence (DA-EPOCH-R):
A single-arm study in young adults used the DA-EPOCH-R regimen (usually six cycles) with filgrastim and no radiation therapy.[93][Level of evidence B4]
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A single-arm study in young adults used the DA-EPOCH-R regimen (usually six cycles) with filgrastim and no radiation therapy.[93][Level of evidence B4]
The 5-year EFS rate was 93%, and the OS rate was 97%.
A single-arm study in young adults used the DA-EPOCH-R regimen (usually six cycles) with filgrastim and no radiation therapy.[93][Level of evidence B4]
At short-term follow-up, there was no evidence of cardiac toxicity, despite a high cumulative dose of doxorubicin for those who received most of the anthracycline-dose escalations.
A single-arm study in young adults used the DA-EPOCH-R regimen (usually six cycles) with filgrastim and no radiation therapy.[93][Level of evidence B4]
An important finding in this study was the prognostic value of end-of-therapy imaging. Nine of 12 patients who had residual mediastinal masses at the end of therapy had positive positron emission tomography scans. Seven of these nine patients had the masses resected, but no viable tumor was found.
A single-arm study in young adults used the DA-EPOCH-R regimen (usually six cycles) with filgrastim and no radiation therapy.[93][Level of evidence B4]
A concern for using this regimen is the significantly higher cumulative doses of alkylating agents and anthracyclines administered than those used in previous regimens.
A multicenter retrospective study of 38 pediatric patients (aged <21 years) and 118 adult patients treated with DA-EPOCH-R observed the following:[94]
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A multicenter retrospective study of 38 pediatric patients (aged <21 years) and 118 adult patients treated with DA-EPOCH-R observed the following:[94]
Pediatric patients had a 3-year EFS rate of 81% and a 3-year OS rate of 91%. These results were not significantly different from the results observed in adults.
A prospective international study included 46 patients (aged <18 years) who were treated with DA-EPOCH-R. The study demonstrated the following results:[95]
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A prospective international study included 46 patients (aged <18 years) who were treated with DA-EPOCH-R. The study demonstrated the following results:[95]
The 4-year EFS rate was 70%, and the 3-year OS rate was 85%.
A prospective international study included 46 patients (aged <18 years) who were treated with DA-EPOCH-R. The study demonstrated the following results:[95]
These outcomes were lower than those in other studies that treated patients with DA-EPOCH-R and were not statistically different from the results of the FAB/LMB-96 (NCT00002757) trial, when a Burkitt lymphoma therapy was used.
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A prospective international study included 46 patients (aged <18 years) who were treated with DA-EPOCH-R. The study demonstrated the following results:[95]
The ability to dose escalate and the adverse events were similar to what has been reported in adult patients.
Evidence (LMB-based chemotherapy plus rituximab):
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96]
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The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96]
The 5-year EFS rate was 88.1% (95% CI, 75%–94.8%) for the whole cohort.
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96] › The 5-year EFS rate was 88.1% (95% CI, 75%–94.8%) for the whole cohort.
The 5-year EFS rate was 81% (95% CI, 60%–92.3%) for patients who did not receive rituximab.
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96] › The 5-year EFS rate was 88.1% (95% CI, 75%–94.8%) for the whole cohort.
The 5-year EFS rate was 95.2% (95% CI, 77.3%–99.2%) for patients who received rituximab.
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96]
The 5-year OS rate was 95.2% for the whole cohort.
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96] › The 5-year OS rate was 95.2% for the whole cohort.
The 5-year OS rate was 90.5% for patients who did not receive rituximab.
The French prospective LMB2001 study reported the outcomes of patients with primary mediastinal B-cell lymphoma who were treated with LMB-based chemotherapy without radiation therapy. There were 773 patients with B-cell NHL, including 42 patients with primary mediastinal B-cell lymphoma, treated between 2001 and 2012. In 2008, the investigators recommended treating all patients with primary mediastinal B-cell lymphoma with rituximab on day 1 of each course. Additionally, patients with bulky mediastinal adenopathy (>10 cm) and/or high LDH serum level (>2N on the Institution upper limit value), and/or lomboaortic nodes were assigned to Group C1 therapy. After 2010, patients with primary mediastinal B-cell lymphoma were treated with a hybrid Group B/C therapy. In total, 21 of 42 patients received rituximab. Nineteen patients were treated with Group B therapy, 18 with Group C therapy, and 5 with Group B/C therapy. The median follow-up was 7.1 years for the entire cohort, 10.6 years for patients who did not receive rituximab, and 6.4 years for patients treated with rituximab.[96] › The 5-year OS rate was 95.2% for the whole cohort.
The 5-year OS rate was 100% for patients who received rituximab.
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