Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion.
Articolo
Data di Pubblicazione:
2011
Abstract:
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive malignancy derived form
precursors of plasmacytoid dendritic cells. We analyzed 21 cases with array-based comparative genomic
hybridization (array-CGH). Complete or partial chromosomal losses largely outnumbered the gains, with
common deleted regions (CDR) 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 Fluorescence In-Situ Hybridization (FISH). This scenario argues for disruption of cell cycle at
G1/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.
Tipologia CRIS:
1.1 Articolo in rivista
Keywords:
blastic plasmacytoid dendritic cell neoplasm; 9p21.3 deletion
Elenco autori:
Lucioni, M; Novara, Francesca; Fiandrino, G; Riboni, R; Fanoni, D; Arra, M; Venegoni, L; Nicola, M; Dallera, E; Arcaini, Luca; Onida, F; Vezzoli, P; Travaglino, E; Boveri, E; Zuffardi, Orsetta; Paulli, Marco; Berti, E.
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