Prognostic relevance of a blastic plasmacytoid dendritic cell neoplasm-like immunophenotype in cytogenetically normal acute myeloid leukemia patients. Issue 7 (6th June 2020)

• Record Type: Journal Article
• Authors: Guolo, Fabio; Minetto, Paola; Clavio, Marino; Marcolin, Riccardo; Miglino, Maurizio; Passannante, Monica; Caviglia, Fabrizio; Ballerini, Filippo; Tedone, Elisabetta; Kunkl, Annalisa; Mangerini, Rosa; Contini, Paola; Colombo, Nicoletta; Cagnetta, Antonia; Cea, Michele; Carminati, Enrico; Pugliese, Girolamo; Gobbi, Marco; Lemoli, Roberto Massimo
• Is Part Of: Leukemia & lymphoma. Volume 61:Issue 7(2020)
• Page Start: 1695
• Page End: 1701
• Publication Date: 2020-06-06
• Subjects: Acute myeloid leukemia -- blastic plasmacytoid dendritic cell neoplasms -- NPM1-mutation -- immunophenotype -- prognosis; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2020.1737685
• Languages: English
• ISSNs: 1042-8194

Abstract

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.