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, and NRAS Mutation 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

FLT3 +

MECOM +

MLF1 +

NPM1 +

RPN1 +

Disease Details

Synonyms
Monomorphic NK-Cell Lymphoma, CD4+/CD56+ Hematodermic Neoplasm, Blastic NK-Cell Lymphoma, BPDCN, Agranular CD4+ Natural Killer Cell Leukemia, Agranular CD4+ CD56+ Hematodermic Neoplasm/Tumor, Blastic Natural Killer Leukemia/Lymphoma
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.