Overview

NCI Definition: A biologic subset of diffuse large B-cell lymphomas with a unique molecular signature or expression profile. It represents approximately 30% of diffuse large B-cell lymphomas, and is characterized by the expression of CD44, PKCbeta1, Cyclin D2, BCL-2, and IRF4/MUM1 genes. Morphologically, these lymphomas are either centroblastic or immunoblastic (ratio 2:1). Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome compared to those with a germinal center B-cell expression profile, with a 5-year survival rate of 35% and a median survival of 2 years. [1]

Diffuse large B-cell lymphoma activated B-cell types most frequently harbor alterations in TP53, MYD88, FGFR4, CSF1R, and CDKN2A [2].

Most Commonly Altered Genes in Diffuse Large B-Cell Lymphoma Activated B-Cell Type

TP53 c.217-c.1178 Missense, TP53 V218L, TP53 V218E, TP53 Mutation, and TP53 Missense are the most common alterations in diffuse large B-cell lymphoma activated B-cell type [2].

Top Alterations in Diffuse Large B-Cell Lymphoma Activated B-Cell Type

Significant Genes in Diffuse Large B-Cell Lymphoma Activated B-Cell Type

AFF1 +

ALK +

BCL2 +

BCL6 +

ELL +

IRF4 +

KMT2A +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

NRIP3 +

Disease Details

Synonyms
ABC DLBCL, Activated B-Cell Type (DLBCL), Activated B-Cell-Like Diffuse Large B-Cell Lymphoma, ABC-DLBCL, Activated B-Cell Type DLBCL, DLBCL Activated B-Cell Type, Diffuse Large B-Cell Lymphoma with an Activated B-Cell Expression Profile
Parent(s)
Diffuse Large B-Cell Lymphoma, Not Otherwise Specified
OncoTree Name
Activated B-Cell Type
OncoTree Code
ABC

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.