Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion.

Abstract:

:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy derived from precursors of plasmacytoid dendritic cells. We analyzed 21 cases with array-based comparative genomic hybridization (aCGH). Complete or partial chromosomal losses largely outnumbered the gains, with common deleted regions involving 9p21.3 (CDKN2A/CDKN2B), 13q13.1-q14.3 (RB1), 12p13.2-p13.1 (CDKN1B), 13q11-q12 (LATS2), and 7p12.2 (IKZF1) regions. CDKN2A/CDKN2B deletion was confirmed by FISH. This scenario argues for disruption of cell cycle at G(1)/S transition, representing a genetic landmark of BPDCN, and possibly contributing to its pathogenesis. Statistical analysis of overall survival in our series highlighted an association of poor outcome with biallelic loss of locus 9p21.3. We suggest that, in the absence of reliable parameters for predicting prognosis in BPDCN other than age, tumor stage, and/or clinical presentation, simple methods, such as FISH for CDKN2A/CDKN2B, could help to identify the most aggressive cases.

journal_name

Blood

journal_title

Blood

authors

Lucioni M,Novara F,Fiandrino G,Riboni R,Fanoni D,Arra M,Venegoni L,Nicola M,Dallera E,Arcaini L,Onida F,Vezzoli P,Travaglino E,Boveri E,Zuffardi O,Paulli M,Berti E

doi

10.1182/blood-2011-03-337501

subject

Has Abstract

pub_date

2011-10-27 00:00:00

pages

4591-4

issue

17

eissn

0006-4971

issn

1528-0020

pii

blood-2011-03-337501

journal_volume

118

pub_type

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