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Primary mediastinal large B-cell lymphoma (PMBCL) is a specific subtype of diffuse large B-cell lymphoma (DLBCL) that accounts for 2% to 4% of non-Hodgkin lymphomas and tends to occur in young women
.
There is no international consensus
on the optimal treatment strategy for PMBCL.
The current regimens commonly used for PMBCL are R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) and DA-EPOCH-R (dose-adjusted etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone, rituximab) regimens, but a significant proportion of patients still require radiotherapy, especially those
treated with R-CHOP regimens 。 Some scholars have explored the efficacy of DA-EPOCH-R regimen in children with PMBCL, but the results are not satisfactory, and the survival rate of children treated with DA-EPOCH-R regimen is lower
than that of adult patients.
The LMB2001 trial, which included ≤ 18-year-old mature B-cell lymphoma (including PMBCL), was designed to investigate the efficacy
of LMB-containing chemotherapy in mature B-cell lymphoma in children/adolescents.
This article reports the results of the LMB2001 trial in
42 patients with PMBCL.
Research methods
All PMBCL patients received 4 to 8 courses of LMB-containing chemotherapy, with rituximab added before each chemotherapy session since 2008
.
The specific treatment plan for PMBCL patients is shown in Table 1, in which group B is stage III and non-central nervous system (CNS) stage IV patients, and the bone marrow involvement < 25%; Group C1 was a stage IV patient with bone marrow involvement ≥ 25% and no CNS-positive disease; Group C3 is CNS-positive).
The primary efficacy endpoint was event-free survival (EFS) and the secondary efficacy endpoint was overall survival (OS).
Table 1
Research results
Baseline characteristics of the patient
In the LMB2001 trial, a total of 42 patients were registered as large B-cell lymphoma with the mediastinum as the main site, and the median age at diagnosis of these patients was 15 years (range: 8-18), 57% were female, and the baseline characteristics of the patients were shown in Table 2
.
Table 2
efficacy
All patients received LMB-containing chemotherapy, including 19 patients in group B, 18 patients in group C, and 5 patients
in group B/C with modified LMB therapy.
After 2008, 21 patients received rituximab (R+) and 21 patients did not receive rituximab (R-).
The median follow-up was 7.
1 years (IQR: 5.
8-11.
1) for all patients, 10.
6 years for R- patients, and 6.
4 years
for R+ patients 。 A total of 5 events occurred, 1 with inadequate response and viable cells in residual masses (R- patient with complete remission [CR] after R-ICE [rituximab, ifosfamide, carboplatin, etoposide], BEAM [carmustine, etoposide, cytarabine, melphalan], autologous hematopoietic stem cell transplantation [ASCT], and radiation therapy [CR] and 3 patients with disease progression (2 R-, 1 R+), and 1 relapse (R-, Patients obtain CR2 and maintain it) (Table 3).
Table 3
A total of 2 patients died, both of which progressed
during rituximab therapy.
The 5-year EFS rate was 88.
1% (95% CI: 75.
0-94.
8) for all patients (Figure 1), 81.
0% (95% CI: 60.
0-92.
3) for R- patients, and 95.
2% (95% CI: 77.
3-99.
2) for R+ patients (HR: 0.
24, 95% CI: 0.
03-2.
2) (Figure 2A).
。 The 5-year OS rate was 95.
2% (95% CI: 84.
0-98.
7) for all patients (Figure 1), 90.
5% (95% CI: 71.
1-97.
3) for R- patients, and 100% for R+ patients (Figure 2B).
Figure 1
Figure 2
Conclusion of the study
The above results show that rituximab + LMB chemotherapy has good efficacy in PMBCL patients aged ≤ 18 years, with a 5-year EFS rate of 95.
2% and a 5-year OS rate of 100%
in R+ patients.
However, the small size of this study and the comparison between patients undergoing LMB chemotherapy have certain limitations, and further prospective, international trials are needed in the future to validate the results of this study and determine the best treatment for
PMBCL patients of all ages.
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.
2022 Sep 1; 107(9):2173-2182.
doi: 10.
3324/haematol.
2021.
280257.
Editor: Wenting Review: Quinta Typesetting: Wenting Execution: moly
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