| Autor: |
Mato, Sara, Castrejón-de-Anta, Natalia, Colmenero, Ariadna, Carità, Lorenzo, Salmerón-Villalobos, Julia, Ramis-Zaldivar, Joan Enric, Nadeu, Ferran, Garcia, Noelia, Wang, Luojun, Verdú-Amorós, Jaime, Andrés, Mara, Conde, Nuria, Celis, Verónica, Ortega, Maria José, Galera, Ana, Astigarraga, Itziar, Perez-Alonso, Vanesa, Quiroga, Eduardo, Jiang, Aixiang, Scott, David W. |
| Předmět: |
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| Zdroj: |
Blood Cancer Journal; 10/7/2024, Vol. 14 Issue 1, p1-10, 10p |
| Abstrakt: |
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. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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