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Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation

ABSTRACT: This SuperSeries is composed of the SubSeries listed below.

ORGANISM(S): Homo sapiens

PROVIDER: GSE116527 | GEO | 2019/02/19

REPOSITORIES: GEO

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Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [ONCOSCAN]

Project description:Burkitt-like lymphoma with 11q aberration (BLL-11q) is a new provisional category in the 2016 WHO classification. The limited number of reported cases does not allow to define whether this is a specific entity or it is a particular variant of other recognized categories such as Burkitt or diffuse large B-cell lymphoma. The genetic alterations involved in the pathogenesis of BLL-11Q are not known.

2019-02-19 | GSE116526 | GEO

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [CYTOSCAN]

2019-02-19 | GSE116522 | GEO

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [SNP6]

2019-02-19 | GSE116518 | GEO

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

Project description: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 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 BTG2, DDX3X, ETS1, 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.

| S-EPMC6717587 | biostudies-literature

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation

Project description:Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation

| PRJNA478946 | ENA

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [CYTOSCAN]

Project description:Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [CYTOSCAN]

| PRJNA478928 | ENA

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [ONCOSCAN]

Project description:Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [ONCOSCAN]

| PRJNA478933 | ENA

Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [SNP6]

Project description:Burkitt-like lymphoma with 11q aberration: A germinal center derived lymphoma without ID3-TCF3-CCND3 pathway deregulation [SNP6]

| PRJNA478920 | ENA

The mutational landscape of Burkitt-like lymphoma with 11q aberration is distinct from that of Burkitt lymphoma.

Project description:The new recently described provisional lymphoma category Burkitt-like lymphoma with 11q aberration comprises cases similar to Burkitt lymphoma (BL) on morphological, immunophenotypic and gene-expression levels but lacking the IG-MYC translocation. They are characterized by a peculiar imbalance pattern on chromosome 11, but the landscape of mutations is not yet described. Thus, we investigated 15 MYC-negative Burkitt-like lymphoma with 11q aberration (mnBLL,11q,) cases by copy-number analysis and whole-exome sequencing. We refined the regions of 11q imbalance and identified the INO80 complex-associated gene NFRKB as a positional candidate in 11q24.3. Next to recurrent gains in 12q13.11-q24.32 and 7q34-qter as well as losses in 13q32.3-q34, we identified 47 genes recurrently affected by protein-changing mutations (each ?3 of 15 cases). Strikingly, we did not detect recurrent mutations in genes of the ID3-TCF3 axis or the SWI/SNF complex that are frequently altered in BL, or in genes frequently mutated in germinal center-derived B-cell lymphomas like KMT2D or CREBBP An exception is GNA13, which was mutated in 7 of 15 cases. We conclude that the genomic landscape of mnBLL,11q, differs from that of BL both at the chromosomal and mutational levels. Our findings implicate that mnBLL,11q, is a lymphoma category distinct from BL at the molecular level.

| S-EPMC6396176 | biostudies-literature

The 11q-Gain/Loss Aberration Occurs Recurrently in MYC-Negative Burkitt-like Lymphoma With 11q Aberration, as Well as MYC-Positive Burkitt Lymphoma and MYC-Positive High-Grade B-Cell Lymphoma, NOS.

Project description:ObjectivesThe latest revision of lymphoma's World Health Organization classification describes the new provisional entity "Burkitt-like lymphoma with 11q aberration" (BLL, 11q) as lacking MYC rearrangement, but harboring the specific11q-gain/loss aberration. We report genetic characteristics of 11 lymphoma cases with this aberration.MethodsClassical cytogenetics, fluorescence in situ hybridization (FISH), and single nucleotide polymorphism/array comparative genomic hybridization.ResultsThe 11q aberrations were described as duplication, inversion, and deletion. Array comparative genomic hybridization showed two types of duplication: bigger than 50 megabase pairs (Mbp) and smaller than 20 Mbp, which were associated with bulky tumor larger than 20 cm and amplification of the 11q23.3 region, including KMT2A. Six cases revealed a normal FISH status of MYC and were diagnosed as BLL,11q. Five cases showed MYC rearrangement and were diagnosed as Burkitt lymphoma (BL) or high-grade B-cell lymphoma, not otherwise specified (HGBL, NOS).ConclusionsThe 11q-gain/loss is not specific for BLL, 11q, but occurs recurrently in MYC-positive BL and MYC-positive HGBL.

| S-EPMC5848380 | biostudies-literature

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