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Primary mediastinal large B-cell lymphoma (PMLBL) is a rare disease that primarily affects adolescents and young adults
.
More recently, an international phase 2 trial of dose-adjusted etoposide, doxorubicin, and cyclophosphamide in combination with vincristine and prednisone plus rituximab (DA-EPOCH-R) in pediatric patients failed to yield positive results.
A French prospective LMB2001 trial included all patients younger than 18 years of age with mature B-cell lymphoma treated at French centers
.
For patients with PMLBL, treatment consists of 4 to 8 cycles of Lymphomes Malins B (LMB)-based chemotherapy without radiation
Figure 1: KaplanMeier estimates of overall survival and event-free survival
.
EFS, event-free survival; OS, overall survival The vertical line represents the Rothman 95% confidence interval
7.
Figure 2: KaplanMeier estimates of event-free survival and overall survival based on rituximab dosing panel A, representing event-free survival
.
Panel B represents overall survival
Figure 2: KaplanMeier estimates of event-free survival and overall survival based on rituximab dosing panel A, representing event-free survival
The 5-year EFS was 81.
Only 1/21 of patients receiving rituximab and LMB-based chemotherapy experienced local early treatment failure, but second-line chemotherapy and radiation prolonged complete remission
Original source:
Original credit: Dourthe ME, Phulpin A, Auperin A, Bosq J, Couec ML, Dartigues P, Ducassou S, Garnier N, Haouy S, Leblanc T, Leruste A, Paillard C, Rigaud C, Simonin M, Patte C, Minard- Colin V.Rituximab in addition to LMB-based chemotherapy regimen in children and adolescents with primary mediastinal large B-cell lymphoma: results of the French LMB2001 prospective study.
Haematologica; https://doi.
org/10.
3324/haematol.
2021.
280257 [Early view] .
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