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Haematologica. 2019 Sep;104(9):1822-1829. doi: 10.3324/haematol.2018.207928. Epub 2019 Feb 7.

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

Author information

1
Hematopathology Unit, Hospital Clínic de Barcelona, University of Barcelona, Barcelona, Spain.
2
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
3
Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain.
4
Pediatric Oncology Department, Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain.
5
Pediatric Oncology Department, Hospital Clínico Universitario de Valencia, Valencia, Spain.
6
Pediatric Oncology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
7
Pathology Department, Hospital Clínico de Valencia, Valencia, Spain.
8
Pathology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
9
Pathology Department, Complejo Hospitalario de Navarra, Pamplona, Spain.
10
Pediatric Hematology Department, Hospital Materno Infantil de Badajoz, Badajoz, Spain.
11
Department of Hematology, Clínica Universidad de Navarra and Instituto de Investigación Sanitaria de Navarra (IdiSNA), Pamplona, Spain.
12
Pediatrics Department, Hospital Universitario Cruces, IIS Biocruces Bizkaia, UPV/EHU, Barakaldo, Spain.
13
Pediatric Oncology Department, Hospital La Fe, Valencia, Spain.
14
Hematopathology Section, Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
15
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain isalaver@clinic.cat.

Abstract

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.

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