Overview

NCI Definition: An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid monocyte. Patients present with cutaneous tumors and bone marrow involvement. [1]

Blastic plasmacytoid dendritic cell neoplasms most frequently harbor alterations in TET2, ASXL1, and NRAS [2].

Most Commonly Altered Genes in Blastic Plasmacytoid Dendritic Cell Neoplasm

TET2 Mutation, TET2 Frameshift, ASXL1 Mutation, NRAS Mutation (somatic), and NRAS Mutation (germline) are the most common alterations in blastic plasmacytoid dendritic cell neoplasm [2].

Top Alterations in Blastic Plasmacytoid Dendritic Cell Neoplasm

Significant Genes in Blastic Plasmacytoid Dendritic Cell Neoplasm

ABL1 +

AFF1 +

BCR +

DEK +

ELL +

FLT3 +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

NPM1 +

NUP214 +

PBX1 +

RPN1 +

TCF3 +

TP53 +

Disease Details

Synonyms
Agranular CD4+ Natural Killer Cell Leukemia, Blastic NK-Cell Lymphoma, CD4+/CD56+ Hematodermic Neoplasm, Agranular CD4+ CD56+ Hematodermic Neoplasm/Tumor, Blastic Natural Killer Leukemia/Lymphoma, Monomorphic NK-Cell Lymphoma, BPDCN
Parent(s)
Malignant Skin Neoplasm
OncoTree Name
Blastic Plasmacytoid Dendritic Cell Neoplasm
OncoTree Code
BPDCN

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 6. 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.