Overview

NCI Definition: A rare B-cell non-Hodgkin lymphoma that is characterized by the abnormal rearrangement of MYC gene, BCL2 gene, and/or BCL6 gene. Patients with this type of lymphoma usually respond poorly to standard treatments and have a poor prognosis. [1]

High grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangementss most frequently harbor alterations in CREBBP, SETD1B, BCL6, KMT2D, and CDKN2A [2].

Most Commonly Altered Genes in High Grade B-Cell Lymphoma with MYC and BCL2 and/or BCL6 Rearrangements

TP53 c.217-c.1178 Missense, TP53 Mutation, TP53 Missense, TET3 V1085A, and SETDB2 Loss are the most common alterations in high grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements [2].

Top Alterations in High Grade B-Cell Lymphoma with MYC and BCL2 and/or BCL6 Rearrangements

Significant Genes in High Grade B-Cell Lymphoma with MYC and BCL2 and/or BCL6 Rearrangements

ALK +

BCL2 +

BCL6 +

IRF4 +

MYC +

Disease Details

Synonyms
High-Grade B-Cell Lymphoma, with MYC and BCL2 and/or BCL6 Rearrangements, HGBL with MYC and BCL2 and/or BCL6 Rearrangements
Parent(s)
Diffuse Large B-Cell Lymphoma
Children
Double-Hit Lymphoma and Triple-Hit Lymphoma
OncoTree Name
High-Grade B-Cell Lymphoma, with MYC and BCL2 and/or BCL6 Rearrangements
OncoTree Code
HGBCLMYCBCL2

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.