Multilineage dysplasia has no impact on biologic, clinicopathologic, and prognostic features of AML with mutated nucleophosmin (NPM1).
Standard
Multilineage dysplasia has no impact on biologic, clinicopathologic, and prognostic features of AML with mutated nucleophosmin (NPM1). / Falini, Brunangelo; Macijewski, Katja; Weiss, Tamara; Bacher, Ulrike; Schnittger, Susanne; Kern, Wolfgang; Kohlmann, Alexander; Klein, Hans-Ulrich; Vignetti, Marco; Piciocchi, Alfonso; Fazi, Paola; Martelli, Maria Paola; Vitale, Antonella; Pileri, Stefano; Miesner, Miriam; Santucci, Antonella; Haferlach, Claudia; Mandelli, Franco; Haferlach, Torsten.
In: BLOOD, Vol. 115, No. 18, 18, 2010, p. 3776-3786.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - Multilineage dysplasia has no impact on biologic, clinicopathologic, and prognostic features of AML with mutated nucleophosmin (NPM1).
AU - Falini, Brunangelo
AU - Macijewski, Katja
AU - Weiss, Tamara
AU - Bacher, Ulrike
AU - Schnittger, Susanne
AU - Kern, Wolfgang
AU - Kohlmann, Alexander
AU - Klein, Hans-Ulrich
AU - Vignetti, Marco
AU - Piciocchi, Alfonso
AU - Fazi, Paola
AU - Martelli, Maria Paola
AU - Vitale, Antonella
AU - Pileri, Stefano
AU - Miesner, Miriam
AU - Santucci, Antonella
AU - Haferlach, Claudia
AU - Mandelli, Franco
AU - Haferlach, Torsten
PY - 2010
Y1 - 2010
N2 - NPM1-mutated acute myeloid leukemia (AML) is a provisional entity in the 2008 World Health Organization (WHO) classification of myeloid neoplasms. The significance of multilineage dysplasia (MLD) in NPM1-mutated AML is unclear. Thus, in the 2008 WHO classification, NPM1-mutated AML with MLD is classified as AML with myelodysplasia (MD)-related changes (MRCs). We evaluated morphologically 318 NPM1-mutated AML patients and found MLD in 23.3%. Except for a male predominance and a lower fms-related tyrosine kinase 3-internal tandem duplication (FLT3-ITD) incidence in the MLD(+) group, no differences were observed in age, sex, cytogenetics, and FLT3--tyrosine kinase domain between NPM1-mutated AML with and without MLD. NPM1-mutated AML with and without MLD showed overlapping immunophenotype (CD34 negativity) and gene expression profile (CD34 down-regulation, HOX genes up-regulation). Moreover, overall and event-free survival did not differ among NPM1-mutated AML patients independently of whether they were MLD(+) or MLD(-), the NPM1-mutated/FLT3-ITD negative genotype showing the better prognosis. Lack of MLD impact on survival was confirmed by multivariate analysis that highlighted FLT3-ITD as the only significant prognostic parameter in NPM1-mutated AML. Our findings indicate that NPM1 mutations rather than MLD dictate the distinctive features of NPM1-mutated AML. Thus, irrespective of MLD, NPM1-mutated AML represents one disease entity clearly distinct from AML with MRCs.
AB - NPM1-mutated acute myeloid leukemia (AML) is a provisional entity in the 2008 World Health Organization (WHO) classification of myeloid neoplasms. The significance of multilineage dysplasia (MLD) in NPM1-mutated AML is unclear. Thus, in the 2008 WHO classification, NPM1-mutated AML with MLD is classified as AML with myelodysplasia (MD)-related changes (MRCs). We evaluated morphologically 318 NPM1-mutated AML patients and found MLD in 23.3%. Except for a male predominance and a lower fms-related tyrosine kinase 3-internal tandem duplication (FLT3-ITD) incidence in the MLD(+) group, no differences were observed in age, sex, cytogenetics, and FLT3--tyrosine kinase domain between NPM1-mutated AML with and without MLD. NPM1-mutated AML with and without MLD showed overlapping immunophenotype (CD34 negativity) and gene expression profile (CD34 down-regulation, HOX genes up-regulation). Moreover, overall and event-free survival did not differ among NPM1-mutated AML patients independently of whether they were MLD(+) or MLD(-), the NPM1-mutated/FLT3-ITD negative genotype showing the better prognosis. Lack of MLD impact on survival was confirmed by multivariate analysis that highlighted FLT3-ITD as the only significant prognostic parameter in NPM1-mutated AML. Our findings indicate that NPM1 mutations rather than MLD dictate the distinctive features of NPM1-mutated AML. Thus, irrespective of MLD, NPM1-mutated AML represents one disease entity clearly distinct from AML with MRCs.
M3 - SCORING: Zeitschriftenaufsatz
VL - 115
SP - 3776
EP - 3786
JO - BLOOD
JF - BLOOD
SN - 0006-4971
IS - 18
M1 - 18
ER -