ACUTE MYELOID LEUKEMIA (AML)

AML is a clonal malignancy of myeloid bone marrow precursors in which poorly differentiated cells accumulate in the bone marrow and circulation.

Signs and symptoms occur because of the absence of mature cells normally produced by the bone marrow, including granulocytes (susceptibility to infection) and platelets (susceptibility to bleeding). In addition, if large numbers of immature malignant myeloblasts circulate, they may invade organs and rarely produce dysfunction. There are distinct morphologic subtypes (Table 66-1) that have largely overlapping clinical features. Of note is the propensity of pts with acute promyelocytic leukemia (APL) (FAB M3) to develop bleeding and disseminated intravascular coagulation, especially during induction chemotherapy, because of the release of procoagulants from their cytoplasmic granules.

TABLE 66-1: AML Classification Systems
World Health Organization classification^a
AML with recurrent genetic abnormalities
AML with t(8;21)(q22;q22); RUNX1-RUNX1T1^b
AML with inv(16)(p13.1;1q22) or t(16;16)(p13.1;q22); CBFB-MYH11^b
Acute promyelocytic leukemia with t(15;17)(q22;q12); PML-RARA^b
AML with t(9;11)(p22;q23); MLLT3-MLL
AML with t(6;9)(p23;q34); DEK-NUP214
AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1
AML (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1
Provisional entity: AML with mutated NPM1
Provisional entity: AML with mutated CEBPA
AML with myelodysplasia-related changes
*Therapy-related myeloid neoplasms*
AML not otherwise specified
AML with minimal differentiation
AML without maturation
AML with maturation
Acute myelomonocytic leukemia
Acute monoblastic and monocytic leukemia
Acute erythroid leukemia
Acute megakaryoblastic leukemia
Acute basophilic leukemia
Acute panmyelosis with myelofibrosis
Myeloid sarcoma
Myeloid proliferations related to Down syndrome
Transient abnormal myelopoiesis
Myeloid leukemia associated with Down syndrome
Blastic plasmacytoid dendritic cell neoplasm
*Acute leukemia of ambiguous lineage*
Acute undifferentiated leukemia
Mixed phenotype acute leukemia with t(9;22)(q34;q11,20); BCR-ABL11
Mixed phenotype acute leukemia with t(v;11q23); MLL rearranged
Mixed phenotype acute leukemia, B/myeloid, NOS
Mixed phenotype acute leukemia, T/myeloid, NOS
Provisional entity: Natural killer (NK)-cell lymphoblastic leukemia/lymphoma
French-American-British (FAB) classification^c
MO: Minimally differentiated leukemia
Ml: Myeloblastic leukemia without maturation
M2: Myeloblastic leukemia with maturation
M3: Hypergranular promyelocytic leukemia
M4: Myelomonocytic leukemia
M4Eo: Variant: Increase in abnormal marrow eosinophils
M5: Monocytic leukemia
M6: Erythroleukemia (DiGuglielmo’s disease)
M7: Megakaryoblastic leukemia

^aData from Swerdlow SH et al (eds): World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, 4th edition update, IARC Press, 2017.

^bDiagnosis is AML regardless of blast count.

^cData from Bennett JM et al: Proposed revised criteria for the classification of acute myeloid leukemia. Ann Intern Med 103:620, 1985.

Abbreviation: AML, acute myeloid leukemia.

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