Multilineage dysplasia does not influence prognosis in CEBPA-mutated AML, supporting the WHO proposal to classify these patients as a unique entity.
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Multilineage dysplasia does not influence prognosis in CEBPA-mutated AML, supporting the WHO proposal to classify these patients as a unique entity. / Bacher, Ulrike; Schnittger, Susanne; Macijewski, Katja; Grossmann, Vera; Kohlmann, Alexander; Alpermann, Tamara; Kowarsch, Andreas; Nadarajah, Niroshan; Kern, Wolfgang; Haferlach, Claudia; Haferlach, Torsten.
In: BLOOD, Vol. 119, No. 20, 20, 2012, p. 4719-4722.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Multilineage dysplasia does not influence prognosis in CEBPA-mutated AML, supporting the WHO proposal to classify these patients as a unique entity.
AU - Bacher, Ulrike
AU - Schnittger, Susanne
AU - Macijewski, Katja
AU - Grossmann, Vera
AU - Kohlmann, Alexander
AU - Alpermann, Tamara
AU - Kowarsch, Andreas
AU - Nadarajah, Niroshan
AU - Kern, Wolfgang
AU - Haferlach, Claudia
AU - Haferlach, Torsten
PY - 2012
Y1 - 2012
N2 - In 2008, the World Health Organization introduced CEBPA (encoding the CCAAT/enhancer binding protein)-mutated acute myeloid leukemia (AML) as a provisional entity. However, the classification of CEBPA-mutated AML with multilineage dysplasia (MLD; ? 50% dysplastic cells in 2-3 lineages) remains to be clarified. In the present study, we investigated 108 CEBPA-mutated AML patients for the impact of MLD, karyotype, and additional mutations. MLD(+) patients differed from MLD(-) patients only by lower mean WBC counts, not by biologic characteristics, cytogenetic risk profiles, or additional mutations. Survival was better for female patients, patients < 60 years of age, for intermediate versus adverse karyotypes, and, in the case of FLT3-ITD negativity, biallelic versus monoallelic/homozygous CEBPA mutations. In contrast, 2-year overall survival and event-free survival did not differ significantly between MLD(+) and MLD(-) patients. By univariable Cox regression analysis, sex, age, WBC count, and cytogenetic risk category were related to overall survival, but MLD was not. Therefore, because dysplasia is not relevant for this subtype, CEBPA-mutated AML patients should be characterized only according to mutation status, cytogenetic risk group, or additional mutations.
AB - In 2008, the World Health Organization introduced CEBPA (encoding the CCAAT/enhancer binding protein)-mutated acute myeloid leukemia (AML) as a provisional entity. However, the classification of CEBPA-mutated AML with multilineage dysplasia (MLD; ? 50% dysplastic cells in 2-3 lineages) remains to be clarified. In the present study, we investigated 108 CEBPA-mutated AML patients for the impact of MLD, karyotype, and additional mutations. MLD(+) patients differed from MLD(-) patients only by lower mean WBC counts, not by biologic characteristics, cytogenetic risk profiles, or additional mutations. Survival was better for female patients, patients < 60 years of age, for intermediate versus adverse karyotypes, and, in the case of FLT3-ITD negativity, biallelic versus monoallelic/homozygous CEBPA mutations. In contrast, 2-year overall survival and event-free survival did not differ significantly between MLD(+) and MLD(-) patients. By univariable Cox regression analysis, sex, age, WBC count, and cytogenetic risk category were related to overall survival, but MLD was not. Therefore, because dysplasia is not relevant for this subtype, CEBPA-mutated AML patients should be characterized only according to mutation status, cytogenetic risk group, or additional mutations.
KW - Adult
KW - Humans
KW - Male
KW - Aged
KW - Female
KW - Middle Aged
KW - Aged, 80 and over
KW - Adolescent
KW - Young Adult
KW - Prognosis
KW - Research Design
KW - Retrospective Studies
KW - Terminology as Topic
KW - Mutation/physiology
KW - Neoplasm Staging/methods
KW - CCAAT-Enhancer-Binding Proteins/genetics
KW - Cell Lineage/genetics
KW - Leukemia, Myeloid, Acute/classification/diagnosis/genetics/pathology
KW - World Health Organization/organization & administration
KW - Adult
KW - Humans
KW - Male
KW - Aged
KW - Female
KW - Middle Aged
KW - Aged, 80 and over
KW - Adolescent
KW - Young Adult
KW - Prognosis
KW - Research Design
KW - Retrospective Studies
KW - Terminology as Topic
KW - Mutation/physiology
KW - Neoplasm Staging/methods
KW - CCAAT-Enhancer-Binding Proteins/genetics
KW - Cell Lineage/genetics
KW - Leukemia, Myeloid, Acute/classification/diagnosis/genetics/pathology
KW - World Health Organization/organization & administration
M3 - SCORING: Journal article
VL - 119
SP - 4719
EP - 4722
JO - BLOOD
JF - BLOOD
SN - 0006-4971
IS - 20
M1 - 20
ER -
