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.

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

Guolo F.;Minetto P.;Clavio M.;Marcolin R.;Miglino M.;Passannante M.;Ballerini F.;Tedone E.;Kunkl A.;Mangerini R.;Contini P.;Colombo N.;Cagnetta A.;Cea M.;Carminati E.;Pugliese G.;Gobbi M.;Lemoli R. M.
2020-01-01

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.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11567/1096498
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