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

Blood. 2019 Feb 28;133(9):962-966. doi: 10.1182/blood-2018-07-864025. Epub 2018 Dec 19.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • ATPases Associated with Diverse Cellular Activities
  • Adolescent
  • Adult
  • Biomarkers, Tumor / genetics*
  • Burkitt Lymphoma / classification*
  • Burkitt Lymphoma / genetics*
  • Burkitt Lymphoma / pathology
  • Child
  • Child, Preschool
  • Chromosome Aberrations*
  • Chromosomes, Human, Pair 11 / genetics*
  • DNA Helicases / genetics
  • DNA-Binding Proteins / genetics
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Mutation*
  • Prognosis
  • Proto-Oncogene Proteins c-myc / genetics
  • Retrospective Studies
  • Young Adult


  • Biomarkers, Tumor
  • DNA-Binding Proteins
  • MYC protein, human
  • NFRKB protein, human
  • Proto-Oncogene Proteins c-myc
  • ATPases Associated with Diverse Cellular Activities
  • DNA Helicases
  • INO80 protein, human