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

Marco Lucioni, Francesca Novara, Giacomo Fiandrino, Roberta Riboni, Daniele Fanoni, Mariarosa Arra, Luigia Venegoni, Marta Nicola, Elena Dallera, Luca Arcaini, Francesco Onida, Pamela Vezzoli, Erica Travaglino, Emanuela Boveri, Orsetta Zuffardi, Marco Paulli, Emilio Berti

Research output: Contribution to journalArticle

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 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.

Original languageEnglish
Pages (from-to)4591-4594
Number of pages4
JournalBlood
Volume118
Issue number17
DOIs
Publication statusPublished - Oct 27 2011

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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