Prognostic relevance of a blastic plasmacytoid dendritic cell neoplasm-like immunophenotype in cytogenetically normal acute myeloid leukemia patients

Fabio Guolo, Paola Minetto, Marino Clavio, Riccardo Marcolin, Maurizio Miglino, Monica Passannante, Fabrizio Caviglia, Filippo Ballerini, Elisabetta Tedone, Annalisa Kunkl, Rosa Mangerini, Paola Contini, Nicoletta Colombo, Antonia Cagnetta, Michele Cea, Enrico Carminati, Girolamo Pugliese, Marco Gobbi, Roberto Massimo Lemoli

Research output: Contribution to journalArticle

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a poor prognosis myeloid malignancy characterized by an atypical phenotype (CD123+, CD56+, and CD4+). We reported that BPDCN-like phenotype (CD123+ and either CD56+ or CD4+ or both) confers poor prognosis to acute myeloblastic leukemia (AML) patients with mutated NPM1. Here, we evaluated the incidence and the prognostic relevance of BPDCN-like phenotype in cytogenetically normal AML (CN-AML) patients. From 2006 to 2016, 83 young (age <60 yrs), consecutive, CN-AML patients underwent intensive treatment. Fifteen patients (18%) showed a BPDCN-like phenotype with no difference between NPM1-mutated (mut) and NPM1-wt patients. It did not significantly affect survival neither in the whole cohort, nor in NPM1-wt patients. However, as reported, it conferred a dismal prognosis in NPM1-mut AML (p < 0.001), irrespectively of the mutational status for FLT3-ITD. In conclusion we show that BPDCN-like phenotype displays a negative prognostic relevance only in NPM1-mutated AML.

Original languageEnglish
JournalLeukemia and Lymphoma
DOIs
Publication statusAccepted/In press - Jan 1 2020

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Keywords

  • Acute myeloid leukemia
  • blastic plasmacytoid dendritic cell neoplasms
  • immunophenotype
  • NPM1-mutation
  • prognosis

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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