MYC-rearranged mature B-cell lymphomas in children and young adults are molecularly Burkitt Lymphoma

MYC-rearranged mature B-cell lymphomas in children and young adults are molecularly Burkitt Lymphoma

Abstract Aggressive B-cell non-Hodgkin lymphomas (NHL) in children, adolescents, and young adults (CAYA) include Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), and a subset of high-grade tumors with features intermediate between these entities whose genetic and molecular profiles have...

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Main Authors: Sara Mato, Natalia Castrejón-de-Anta, Ariadna Colmenero, Lorenzo Carità, Julia Salmerón-Villalobos, Joan Enric Ramis-Zaldivar, Ferran Nadeu, Noelia Garcia, Luojun Wang, Jaime Verdú-Amorós, Mara Andrés, Nuria Conde, Verónica Celis, Maria José Ortega, Ana Galera, Itziar Astigarraga, Vanesa Perez-Alonso, Eduardo Quiroga, Aixiang Jiang, David W. Scott, Elias Campo, Olga Balagué, Itziar Salaverria
Format: Article
Language:English
Published: Nature Publishing Group 2024-10-01
Series:Blood Cancer Journal
Online Access:https://doi.org/10.1038/s41408-024-01153-0
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Summary:Abstract Aggressive B-cell non-Hodgkin lymphomas (NHL) in children, adolescents, and young adults (CAYA) include Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), and a subset of high-grade tumors with features intermediate between these entities whose genetic and molecular profiles have not been completely elucidated. In this study, we have characterized 37 aggressive B-NHL in CAYA, 33 with high-grade morphology, and 4 DLBCL with MYC rearrangement (MYC-R), using targeted next-generation sequencing and the aggressive lymphoma gene expression germinal center B-cell-like (GCB), activated B-cell-like (ABC), and dark zone signatures (DZsig). Twenty-two tumors had MYC-R without BCL2 breaks, and two MYC-non-R cases had BCL6 translocations. MYC-R cases, including DLBCL, carried BL-related mutations and copy number alterations. Conversely, MYC-non-R lymphomas had alterations in the B-cell receptor signaling/NF-κB pathway (71%). DZsig was expressed in 12/13 of MYC-R tumors but only in 2/10 of MYC-non-R GCB tumors (P < 0.001). The 3-year event-free survival (EFS) of the whole cohort was 79.6%. TP53 and KMT2C mutations conferred inferior outcome (3-year EFS P < 0.05). Overall, MYC-R lymphomas in CAYA have a molecular profile similar to BL regardless of their high-grade or DLBCL morphology, whereas MYC-non-R has more heterogeneous genetic alterations closer to that of DLBCL.
ISSN:2044-5385

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