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

Burkitt-like lymphoma with 11q aberration is characterized by pathological features and gene expression profile resembling those of Burkitt lymphoma but lacks the MYC rearrangement and carries an 11q-arm aberration with proximal gains and telomeric losses. Whether this lymphoma is a distinct categor...

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Bibliographic Details
Authors: González Farre, Blanca, Ramis Zaldivar, Joan Enric, Salmerón Villalobos, Julia, Balagué, Olga, Celis, Verónica, Verdú Amorós, Jaime, Nadeu, Ferran, Sábado, Constantino, Ferrández, Antonio, Garrido, Marta, García Bragado, Federico, De la Maya, María Dolores, Vagace, José Manuel, Panizo, Carlos Manuel, Astigarraga Aguirre, María Iciar, Andrés, Mara, Jaffe, Elaine S., Campo, Elias, Salaverria, Itziar
Format: article
Publication Date:2019
Country:España
Institution:Universidad del País Vasco
Repository:Addi. Archivo Digital para la Docencia y la Investigación
OAI Identifier:oai:addi.ehu.eus:10810/37043
Online Access:http://hdl.handle.net/10810/37043
Access Level:Open access
Keyword:B-cell lymphoma
copy-number
myc
genome
gene
pathogenesis
expression
translocations
abnormalities
mutations
Description
Summary:Burkitt-like lymphoma with 11q aberration is characterized by pathological features and gene expression profile resembling those of Burkitt lymphoma but lacks the MYC 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 lymphomarelated 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 BTG2, DDX3X, ETSI , EP300, and GNA13. However, ID3, TCF3, or CCND3 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.

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