Burkitt-like lymphoma with 11q aberration: a germinal center-derived lymphoma genetically unrelated to Burkitt lymphoma

• Published in: Haematologica (Roma) Vol. 104; no. 9; pp. 1822 - 1829
• Main Authors: Gonzalez-Farre, Blanca, Ramis-Zaldivar, Joan Enric, Salmeron-Villalobos, Julia, Balagué, Olga, Celis, Verónica, Verdu-Amoros, Jaime, Nadeu, Ferran, Sábado, Constantino, Ferrández, Antonio, Garrido, Marta, García-Bragado, Federico, de la Maya, María Dolores, Vagace, José Manuel, Panizo, Carlos Manuel, Astigarraga, Itziar, Andrés, Mara, Jaffe, Elaine S, Campo, Elias, Salaverria, Itziar
• Format: Journal Article
• Language: English
• Published: Italy Ferrata Storti Foundation 01.09.2019
• Subjects: Limfomes; Lymphomas; Oncologia pediàtrica; Tumors in children
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.2018.207928

Burkitt-like lymphoma with 11q aberration is characterized by pathological features and gene expression profile resembling those of Burkitt lymphoma but lacks the rearrangement and carries an 11q-arm aberration with proximal gains and telomeric losses. Whether this lymphoma is a distinct category or a particular variant of other recognized entities is controversial. To improve the understanding of Burkitt-like lymphoma with 11q aberration we performed an analysis of copy number alterations and targeted sequencing of a large panel of B-cell lymphoma-related genes in 11 cases. Most patients had localized nodal disease and a favorable outcome after therapy. Histologically, they were high grade B-cell lymphoma, not otherwise specified (8 cases), diffuse large B-cell lymphoma (2 cases) and only one was considered as atypical Burkitt lymphoma. All cases had a germinal center B-cell signature and phenotype with frequent LMO2 expression. The patients with Burkitt-like lymphoma with 11q aberration had frequent gains of 12q12-q21.1 and losses of 6q12.1-q21, and lacked common Burkitt lymphoma or diffuse large B-cell lymphoma alterations. Potential driver mutations were found in 27 genes, particularly involving , , , , and However, , , or mutations were absent in all cases. These results suggest that Burkitt-like lymphoma with 11q aberration is a germinal center-derived lymphoma closer to high-grade B-cell lymphoma or diffuse large B-cell lymphoma than to Burkitt lymphoma.