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• W2950986696 abstract "Background: Blastic plasmacytoid dendritic cell neoplasms (BPDCN) are rare disorders included by WHO 2016 classification among the myeloid neoplasms and characterized by aggressive clinical course and dismal outcome. BPDCN display a recurrent phenotype (CD45low/CD34-/CD56+/CD4+/CD123+), in the absence of lineage differentiation. Our group recently reported a negative prognostic impact of CD123, CD56 and CD4 co-expression (“BPDCN-like” phenotype) in AML patients with NPM1-mutation. Aims: The aim of the present study was to evaluate the incidence and the prognostic impact of BPDCN-like phenotype in a wider cohort of cytogenetically normal AML patients, irrespectively of NPM1-mutational status. Methods: We retrospectively evaluated a cohort of 83 younger (age <60 yrs), consecutive AMLpatientswith normal karyotype, who have been intensively treated in our institution between 2006 and 2016. All patients were treated with the same fludarabine-idarubicin-Ara-C containing induction. In all patients, four (until 2012) or eight color immunophenotypic analysis has been performed at diagnosis. We defined a BPDCN-like signature as the combination of at least two among CD56+/CD4+/CD123+ and evaluated its prognostic impact as well as the correlation with biological, molecular and cytogenetic features. Results: Fifteen patients (18%) showed a BPDCN-like signature. Neither the presence of NPM1-mutation nor FLT3-ITD or biallelic CEBPA mutation had a significant correlation with BPDCN-like signature. BPDCN-like patients had significantly higher WBC count at diagnosis (p < 0.05). No clear correlation with sex or age at diagnosis was observed. In the whole cohort of patients, only the presence of NPM1-mutation correlated with the achievement of complete response (CR). Median follow-up was 63 months, 3-year Overall Survival (OS) was 52% in the whole cohort (median 38 months). Patient with NPM1 mutation or biallelic CEBPA mutation had a better outcome (p < 0.03). OS was not significantly influenced by the FLT3-ITD mutation or by the presence of BPDCN-like signature. However, as we previously reported, in the subgroup of 30 patients with NPM-1 mut AML, the presence of BPDCN-like features conferred a dismal prognosis (3-year OS 25% vs 77% for BPDCN positive and negative patients, respectively, p < 0.001), irrespectively of mutational status for FLT3-ITD or other clinical features. Even if CR rate was not affected, all NPM1-mut patient with BPDCN-like phenotype failed to achieve minimal residual disease (MRD)-negative CR (p < 0.05). On the contrary the negative prognostic influence of BPDCN-like phenotype was not observed in the 53 NPM-1 wt patients (3-year OS 71% vs 35% for BPDCN-like positive and negative NPM-wt patients, respectively, p = 0.156). No difference in MRD clearance probability was observed in this subgroup.Summary/Conclusion: Our extended analysis confirms that a peculiar BPDCN-like immunophenotype among NPM-1 mut AML patients is associated with significantly worse outcome in this otherwise favorable subset of AML. Patient with BPDCN-like features, even if achieving morphological CR showed high level of minimal residual disease after induction, suggesting an intrinsic chemo-resistance. Interestingly, this observation was strictly restricted to NPM-1 mutated AML, suggesting that peculiar alterations in this distinct entity contribute to the prognostic impact of BPDCN-like features in this setting. The biological explanation of this finding is not clear; further gene-expression profiling studies are ongoing in order to explain our findings." @default.
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• W2950986696 date "2019-06-01" @default.
• W2950986696 modified "2023-09-24" @default.
• W2950986696 title "PF290 INCIDENCE AND PROGNOSTIC IMPACT OF A BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM-LIKE PHENOTYPE IN AML PATIENTS" @default.
• W2950986696 doi "https://doi.org/10.1097/01.hs9.0000559372.40300.8a" @default.
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