A Blastic Plasmacytoid Dendritic Cell Neoplasm-like Phenotype Identifies a Subgroup of NPM1-Mutated AML Patients with Worse Prognosis While Has Not Predictive Value in NPM1-Wt AML

Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) are a very rare group of diseases included by WHO 2016 classification among the myeloid neoplasms and usually display an aggressive course with dismal outcome. BPDCN are characterized by a recurrent phenotype (CD45low/CD34-/CD56+/CD4+/C...

Full description

Saved in:
Bibliographic Details
Published in:Blood 2018-11, Vol.132 (Supplement 1), p.5243-5243
Main Authors: Guolo, Fabio, Minetto, Paola, Kunkl, Annalisa, Tedone, Elisabetta, Clavio, Marino, Miglino, Maurizio, Caviglia, Fabrizio, Colombo, Nicoletta, Cagnetta, Antonia, Cea, Michele, Ballerini, Filippo, Ravetti, Jean Louis, Gobbi, Marco, Lemoli, Roberto Massimo
Format: Article
Language:English
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) are a very rare group of diseases included by WHO 2016 classification among the myeloid neoplasms and usually display an aggressive course with dismal outcome. BPDCN are characterized by a recurrent phenotype (CD45low/CD34-/CD56+/CD4+/CD123+), in the absence of other lineage differentiation markers. Our group recently reported that a subset of patients diagnosed with AML with NPM1-mutation carrying co-expression of CD123, CD56 and CD4, a “BPDCN-like” phenotype, showed poor prognosis. The aim of the present study was to evaluate the incidence and the prognostic impact of BPDCN-like phenotype in a wider cohort of cytogenetically normal AML patients, irrespectively of NPM1-mutational status. Methods We retrospectively evaluated a cohort of 83 younger (age
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2018-99-118984