Figure 1.
Hierarchical classification of the International Consensus Classification of AML. The classification is hierarchical (ie, AML with recurrent genetic abnormalities takes precedence over all other categories); among the remaining categories, AML with mutated TP53 supersedes AML with myelodysplasia-related gene mutations, and the latter supersedes AML with myelodysplasia-related cytogenetic abnormalities. aMyeloblasts, monoblasts, and megakaryoblasts are included in the blast count. Monoblasts and promonocytes, but not abnormal monocytes, are counted as blast equivalents in AML with monocytic or myelomonocytic differentiation, and promyelocytes in the setting of PML::RARA or variant RARA rearrangement. Cases with prior diagnosis of MPN are excluded and are classified as accelerated (10%-19% blasts) or blast phase (≥20% blasts) MPN. For patients who already have a history of MDS/MPN (eg, CMML), the diagnosis of MDS/MPN should be retained until there are ≥20% blasts/blast equivalents; however, once an AML-defining recurrent genetic abnormality (eg, KMT2A rearrangement or NPM1 mutation) is detected and the blast count is ≥10%, AML-type therapy is recommended. bAML-defining recurrent genetic abnormalities are t(15;17)(q24.1;q21.2)/PML::RARA; t(8;21)(q22;q22.1)/RUNX1::RUNX1T1; inv(16)(p13.1q22) or t(16;16)(p13.1;q22)/CBFB::MYH11; t(9;11)(p21.3;q23.3)/MLLT3::KMT2A; t(6;9)(p22.3;q34.1)/DEK::NUP214; inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2)/GATA2, MECOM(EVI1); mutated NPM1; in-frame bZIP mutated CEBPA; t(9;22)(q34.1;q11.2)/BCR::ABL1; other recurrent rearrangements involving RARA, KMT2A, MECOM, and other rare rearrangements as listed in Table 1. The entity is named with the specific genetic abnormality. Cases with BCR::ABL1 rearrangement and 10% to 19% blasts are classified as CML in accelerated phase, and cases with history of CML and ≥20% blasts are classified as CML in myeloid blast phase. cExamples how to append diagnostic qualifiers: AML with myelodysplasia-related cytogenetic abnormality, therapy-related; AML with myelodysplasia-related gene mutation, prior myelodysplastic syndrome; AML with myelodysplasia-related gene mutation, germline RUNX1 mutation (ie, gene or syndrome should be specified).

Hierarchical classification of the International Consensus Classification of AML. The classification is hierarchical (ie, AML with recurrent genetic abnormalities takes precedence over all other categories); among the remaining categories, AML with mutated TP53 supersedes AML with myelodysplasia-related gene mutations, and the latter supersedes AML with myelodysplasia-related cytogenetic abnormalities. aMyeloblasts, monoblasts, and megakaryoblasts are included in the blast count. Monoblasts and promonocytes, but not abnormal monocytes, are counted as blast equivalents in AML with monocytic or myelomonocytic differentiation, and promyelocytes in the setting of PML::RARA or variant RARA rearrangement. Cases with prior diagnosis of MPN are excluded and are classified as accelerated (10%-19% blasts) or blast phase (≥20% blasts) MPN. For patients who already have a history of MDS/MPN (eg, CMML), the diagnosis of MDS/MPN should be retained until there are ≥20% blasts/blast equivalents; however, once an AML-defining recurrent genetic abnormality (eg, KMT2A rearrangement or NPM1 mutation) is detected and the blast count is ≥10%, AML-type therapy is recommended. bAML-defining recurrent genetic abnormalities are t(15;17)(q24.1;q21.2)/PML::RARA; t(8;21)(q22;q22.1)/RUNX1::RUNX1T1; inv(16)(p13.1q22) or t(16;16)(p13.1;q22)/CBFB::MYH11; t(9;11)(p21.3;q23.3)/MLLT3::KMT2A; t(6;9)(p22.3;q34.1)/DEK::NUP214; inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2)/GATA2, MECOM(EVI1); mutated NPM1; in-frame bZIP mutated CEBPA; t(9;22)(q34.1;q11.2)/BCR::ABL1; other recurrent rearrangements involving RARA, KMT2A, MECOM, and other rare rearrangements as listed in Table 1. The entity is named with the specific genetic abnormality. Cases with BCR::ABL1 rearrangement and 10% to 19% blasts are classified as CML in accelerated phase, and cases with history of CML and ≥20% blasts are classified as CML in myeloid blast phase. cExamples how to append diagnostic qualifiers: AML with myelodysplasia-related cytogenetic abnormality, therapy-related; AML with myelodysplasia-related gene mutation, prior myelodysplastic syndrome; AML with myelodysplasia-related gene mutation, germline RUNX1 mutation (ie, gene or syndrome should be specified).

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