Page 174 - Haematologica Vol. 107 - September 2022

ARTICLE - Non-Hodgkin Lymphoma
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
Marie Emilie Dourthe,1,2 Aurélie Phulpin,3 Anne Auperin,4 Jacques Bosq,5 Marie-Laure Couec,6 Peggy Dartigues,5 Stéphane Ducassou,7 Nathalie Garnier,8 Stéphanie Haouy,9 Thierry Leblanc,2 Amaury Leruste,10 Catherine Paillard,11 Charlotte Rigaud,12 Mathieu Simonin,2,13 Catherine Patte12 and Véronique Minard-Colin12,14
1Department of Pediatric Hematology and Immunology, Robert Debré University Hospital, AP- HP and University de Paris, Paris; 2Université de Paris, Institut Necker-Enfants Malades (INEM), Institut National de la Santé et de la Recherche Médicale, INSERM U1151, and Laboratory of Onco-Hematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker Enfants-Malades, Paris; 3Pediatric Oncology and Hematology Department, University Hospital of Nancy (CHRU Nancy), Nancy; 4Service de Biostatistique et d'Epidémiologie, Gustave Roussy Cancer Campus, INSERM U1018, CESP, Université Paris-Sud, Université Paris-Saclay, Villejuif ; 5Department of Biopathology, Morphology Unit, Gustave Roussy, Université Paris Saclay, Villejuif; 6Department of Pediatric Hematology and Oncology, University Hospital of Nantes, Nantes; 7Department of Pediatric Oncology and Hematology, University Hospital of Bordeaux, Bordeaux; 8Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, Lyon; 9Department of Pediatric Oncology and Hematology, University Hospital of Montpellier, Montpellier; 10SIREDO Oncology Center, Institute Curie, PSL Research University, Paris; 11Department of Pediatric Hematology Oncology, University Hospital of Strasbourg, INSERM UMRS 1109, Strasbourg; 12Department of Pediatric and Adolescent Oncology, Gustave Roussy, Université Paris-Saclay, Villejuif; 13Pediatric Hematology Oncology Department, Sorbonne Université/Trousseau Hospital, AP-HP, Paris and 14Gustave Roussy and Paris-Saclay University, INSERM 1015, Paris, France
Abstract
Primary mediastinal large B-cell lymphoma (PMLBL) is a rare entity predominantly affecting adolescents and young adults. Recently, an international phase II trial in pediatric patients using dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine and prednisone plus rituximab (DA-EPOCH-R) failed to reproduce excellent survival reported in some adult studies. The optimal therapy regimen needs to be determined in this disease. The French prospective LMB2001 trial included all patients ≤18 years with mature B-cell lymphoma treated in French centers. For patients with PMLBL, treatment included four to eight courses of Lymphomes Malins B (LMB)-based chemotherapy without radiotherapy. From 2008, rituximab was added before each chemotherapy course. From 09/2001 to 03/2012, 42 patients with PMLBL were registered. The median age was 15 years (range, 8-18). Twenty-one patients were treated with chemotherapy plus rituximab. The median follow-up was 7.1 years (interquartile range, 5.8-11.1). Five-year event-free and overall survival were 88.1% (95% confidence interval (CI): 75.0-94.8) and 95.2% (95% CI: 84.0-98.7) for the whole population. The 5-year EFS was 81.0% (95% CI: 60.0-92.3) and 95.2% (95% CI: 77.3-99.2) (hazard ratio =0.24; 95% CI: 0.03- 2.2) and 5-year overall survival was 90.5% (95% CI: 71.1-97.3) and 100% for patients treated without and with rituximab, respectively. Only one of 21 patients treated with rituximab and LMB-based chemotherapy had local early treatment failure but achieved prolonged complete remission with second-line chemotherapy and radiotherapy. Intensive LMB- based chemotherapy with rituximab achieved excellent survival in children/adolescents with PMLBL. Further international prospective studies are required to confirm these results in this population.
(PMLBL) is a distinct pathogenetic subtype of mature B-
cell neoplasms.1 It is a rare entity representing 2-4% of Primary mediastinal (thymic) large B-cell lymphoma adult and pediatric non-Hodgkin lymphoma.2,3 PMLBL
Haematologica | 107 September 2022
Correspondence:
M-E. Dourthe
marie-emilie.dourthe@aphp.fr
V. Minard-Colin
veronique.minard@gustaveroussy.fr
Received: Accepted: Prepublished:
October 27, 2021. February 23, 2022. March 3, 2022.
https://doi.org/10.3324/haematol.2021.280257
©2022 Ferrata Storti Foundation Published under a CC BY-NC license
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