Overview

NCI Definition: A clonal neoplasm of small B-lymphocytes, lymphoplasmacytoid cells, and plasma cells involving the bone marrow, lymph nodes, and the spleen. The majority of patients have a serum IgM paraprotein. [1]

Lymphoplasmacytic lymphomas most frequently harbor alterations in MYD88, DNMT3A, TP53, CXCR4, and TET3 [2].

Most Commonly Altered Genes in Lymphoplasmacytic Lymphoma

MYD88 Mutation, MYD88 L265P, MYD88 Exon 5 Mutation, DNMT3A Mutation, and TP53 c.217-c.1178 Missense are the most common alterations in lymphoplasmacytic lymphoma [2].

Top Alterations in Lymphoplasmacytic Lymphoma

Significant Genes in Lymphoplasmacytic Lymphoma

ABL1 +

AFF1 +

BCL2 +

BCL6 +

BCR +

CBFB +

CCND1 +

DEK +

ELL +

FLT3 +

IGH +

IKZF1 +

IRAK1 +

JAK2 +

JAK3 +

KIT +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

MYD88 +

MYH11 +

NPM1 +

NRIP3 +

NUP214 +

PBX1 +

RPN1 +

RUNX1 +

RUNX1T1 +

TCF3 +

TP53 +

TYK2 +

Disease Details

Synonyms
Lymphoma, Plasmacytic, LYMPHOMA, LYMPHOPLASMACYTIC, MALIGNANT, Lymphoplasmacytoid Lymphoma, Immunocytoma, Lymphoplasmacytic Type, Lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia), Waldenstrom macroglobulinemia
Parent(s)
Mature B-Cell Non-Hodgkin Lymphoma
Children
Waldenstrom Macroglobulinemia
OncoTree Name
Lymphoplasmacytic Lymphoma
OncoTree Code
LPL

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.