Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Classification of Pediatric Myeloid Malignancies
In 2008, the WHO expanded the number of cytogenetic abnormalities linked to AML classification, and for the first time included specific gene mutations (CEBPA and NPM mutations) in its classification system. Such a genetically based classification system links AML class with outcome and provides significant biologic and prognostic information. With new emerging technologies aimed at genetic, epigenetic, proteomic, and immunophenotypic classification, AML classification will likely evolve and provide informative prognostic and biologic guidelines to clinicians and researchers.
WHO classification of AML
- AML with recurrent genetic abnormalities:
- AML with t(8;21)(q22;q22), RUNX1-RUNX1T1(CBFA2-AML1-ETO).
- AML with inv(16)(p13.1;q22) or t(16;16)(p13.1;q22), CBFB-MYH11.
- APL with t(15;17)(q24;q21), PML-RARA.
- AML with t(9;11)(p22;q23), MLLT3(AF9)-MLL.
- AML with t(6;9)(p23;q34), DEK-NUP214.
- AML with inv(3)(q21;q26.2) or t(3;3)(q21;q26.2), RPN1-EVI1.
- AML (megakaryoblastic) with t(1;22)(p13;q13), RBM15-MKL1.
- AML with mutated NPM1.
- AML with mutated CEBPA.
- AML with myelodysplasia-related features.
- 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.
The treatment for children with AML differs significantly from that for acute lymphoblastic leukemia (ALL). As a consequence, it is critical to distinguish AML from ALL. Special histochemical stains performed on bone marrow specimens of children with acute leukemia can be helpful to confirm their diagnosis, although such approaches have been mostly replaced by flow cytometric immunophenotyping. The stains most commonly used include myeloperoxidase, periodic acid-Schiff, Sudan Black B, and esterase. In most cases the staining pattern with these histochemical stains will distinguish AML from AMML and ALL (see below).
Table 1. Histochemical Staining Patternsa
|M0||AML, APL (M1-M3)||AMML (M4)||AMoL (M5)||AEL (M6)||AMKL (M7)||ALL|
|AEL = acute erythroid leukemia; ALL = acute lymphoblastic leukemia; AML = acute myeloid leukemia; AMKL = acute megakaryocytic leukemia; AMML = acute myelomonocytic leukemia; AMoL = acute monocytic leukemia; APL = acute promyelocytic leukemia; PAS = periodic acid-Schiff.|
|a Refer to theFrench-American-British (FAB) Classification for Childhood Acute Myeloid Leukemiasection of this summary for more information about the morphologic-histochemical classification system for AML.|
|b These reactions are inhibited by fluoride.|
|Sudan Black B||-||+||+||-||-||-||-|