Page 182 - Haematologica Vol. 107 - September 2022

ARTICLE - Rituximab with LMB-chemotherapy regimen in PMLBL M.E. Dourthe et al.
LMB chemotherapy backbone22 and the total cumulative dose of doxorubicin is limited and of 240 mg/m2 with the LMB B/C-modified combination (favorably compared with the DA-EPOCH-R in the Inter-B-NHL ritux 2010 study where 72% of patients received ≥300 mg/m2 doxorubicin and 24% ≥350 mg/m2)7.
Although our study has some limitations, i.e., i) relative small series of 42 patients but it compares well with other published reports in this rare disease, ii) pathology review for only 85% of cases, iii) no randomized comparison be- tween LMB chemotherapy only and LMB chemotherapy with rituximab, and iv) some differences during the study duration in terms of chemotherapy group recommenda- tions, this was a prospective multicentric study, and we believe that these excellent results are important for the medical community of pediatric, adolescents, and young adults oncologists. Further prospective and international trials are required to confirm these results and define op- timal treatment for patients with PMLBL (all ages in- cluded). Novel agents (e.g., NF-kB pathway inhibitors or anti-PD1 therapies combined or not with brentuximab-ve- dotin) may be required next to reduce chemotherapy in- tensity and improve outcome in this population.23
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Disclosures
No conflicts of interest to disclose.
Contributions
CP, AA, MED, and VM-C conceived the study and oversaw the project; AP, NG, NA, JM, SH, CP, JL-P, TL, CS, CP and VM-C recruited patients; AP, VM-C, AA, CP, JB and MED col- lected and assembled data; AA performed statistical analysis; MED, AP, AA, JB, CP, VM-C analyzed and inter- preted data; MED, AP and VM-C wrote the manuscript. All authors approved the manuscript.
Acknowledgments
The authors thank Yasmina Oubouzar for data manage- ment. We thank all patients and their families for partici- pating in the studies. We thank our colleagues in the hospitals and reference institutions who contributed to this study, for their care for the children and families, and the supplied data.
Data-sharing statement
The data that support the findings of this study are avail- able on request from the corresponding author.
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