Table 1.

Risk status stratification by genetic abnormality per ELN 2017 and NCCN 2017 guidelines

Risk category*ELN criteria10NCCN criteria6
Favorablet(8;21)(q22;q22.1); RUNX1-RUNX1T1Core binding factor: inv(16), or t(16;16), or t(8;21), or t(15;17)
inv(16)(p13.1;q22) or t(16;16)(p13.1;q22); CBFB-MYH11Normal cytogenetics: NPM1 mutation in absence of FLT3-ITD or isolated biallelic (double) CEBPA mutation
Mutated NPM1 without FLT3-ITD or with FLT3-ITDlow§
Biallelic mutated CEBPA
IntermediateMutated NPM1 and FLT3-ITDhigh§Normal cytogenetics
Wild-type NPM1 without FLT3-ITD or with FLT3-ITDlow§ (without adverse-risk genetic lesions)+8 alone
t(9;11)(p21.3;q23.3); MLLT3-KMT2A||t(9;11)
Cytogenetic abnormalities not classified as favorable or adverseOther nondefined
Core binding factor with KIT mutation
Poor/adverset(6;9)(p23;q34.1); DEK-NUP214Complex (≥3 clonal chromosomal abnormalities)
t(v;11q23.3); KMT2A rearrangedMonosomal karyotype
t(9;22)(q34.1;q11.2); BCR-ABL1−5, 5q–, –7, 7q–
inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2,MECOM(EVI1)11q23 – non t(9;11)
−5 or del(5q); –7; –17/abn(17p)inv(3), t(3;3)
Complex karyotype, monosomal karyotype#t(6;9)
Wild-type NPM1 and FLT3-ITDhight(9;22)
Mutated RUNX1**Normal cytogenetics: with FLT3-ITD mutation††
Mutated ASXL1**TP53 mutation
Mutated TP53‡‡
  • * The prognostic value of a marker is treatment-dependent and may change with new therapies.

  • Presence of KIT mutations in patients with t(8:21) and, to a lesser extent, inv(16), confers a high risk of relapse; these patients should be considered intermediate risk and considered for HSCT if available.

  • Other cytogenetic findings in addition to these do not alter risk status.

  • § Low, low allelic ratio (<0.5); high, high allelic ratio (≥0.5); recent studies indicate that AML with NPM1 mutation and FLT3-ITD low allelic ratio may also have a more favorable prognosis and patients should not routinely be assigned to allogeneic HCT.75,76

  • || Presence of t(9;11)(p21.3;q23.3) takes precedence over rare, concurrent adverse-risk gene mutations.

  • 3 or more chromosomal abnormalities in the absence of 1 of the World Health Organization–designated recurring translocations or inversions: t(8;21), inv(16) or t(16;16), t(9;11), t(v;11)(v;q23.3), t(6;9), inv(3) or t(3;3); AML with BCR-ABL1.

  • # Defined by the presence of 1 single monosomy (excluding loss of X or Y) with at least 1 additional monosomy or structural chromosome abnormality (excluding core-binding factor AML).

  • ** These should not be used as adverse prognostic markers if they occur with favorable-risk AML subtypes.

  • †† FLT3-ITD mutations are considered to confer a significantly poorer outcome in patients with normal karyotype; there is controversy about whether FLT3-TKD mutations carry equally poor prognosis.

  • ‡‡ TP53 mutations are significantly associated with AML with complex and monosomal karyotype.

  • NCCN, National Comprehensive Cancer Network.