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
Bloodjournal_title
Bloodauthors
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 Edoi
10.1182/blood-2011-03-337501subject
Has Abstractpub_date
2011-10-27 00:00:00pages
4591-4issue
17eissn
0006-4971issn
1528-0020pii
blood-2011-03-337501journal_volume
118pub_type
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