Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Classification of Pediatric Myeloid Malignancies
French-American-British (FAB) Classification for Childhood Acute Myeloid Leukemia
The first comprehensive morphologic-histochemical classification system for acute myeloid leukemia (AML) was developed by the FAB Cooperative Group.[1,2,3,4,5] This classification system, which has been replaced by the World Health Organization (WHO) system described below, categorized AML into the following major subtypes primarily based on morphology and immunohistochemical detection of lineage markers:
M0: Acute myeloblastic leukemia without differentiation.[6,7] M0 AML, also referred to as minimally differentiated AML, does not express myeloperoxidase (MPO) at the light microscopy level, but may show characteristic granules by electron microscopy. M0 AML can be defined by expression of cluster determinant (CD) markers such as CD13, CD33, and CD117 (c-KIT) in the absence of lymphoid differentiation.
M1: Acute myeloblastic leukemia with minimal differentiation but with the expression of MPO that is detected by immunohistochemistry or flow cytometry.
M2: Acute myeloblastic leukemia with differentiation.
M3: Acute promyelocytic leukemia (APL) hypergranular type. (Refer to the Acute Promyelocytic Leukemia section of this summary for more information on treatment options under clinical evaluation.)
M3v: APL, microgranular variant. Cytoplasm of promyelocytes demonstrates a fine granularity, and nuclei are often folded. Same clinical, cytogenetic, and therapeutic implications as FAB M3.
M4: Acute myelomonocytic leukemia (AMML).
M4Eo: AMML with eosinophilia (abnormal eosinophils with dysplastic basophilic granules).
M5: Acute monocytic leukemia (AMoL).
- M5a: AMoL without differentiation (monoblastic).
- M5b: AMoL with differentiation.
M6: Acute erythroid leukemia (AEL).
- M6a: Erythroleukemia.
- M6b: Pure erythroid leukemia (myeloblast component not apparent).
- M6c: Presence of myeloblasts and proerythroblasts.
M7: Acute megakaryocytic leukemia (AMKL).
Other extremely rare subtypes of AML include acute eosinophilic leukemia and acute basophilic leukemia.
World Health Organization (WHO) Classification System
In 2001, the WHO proposed a new classification system that incorporated diagnostic cytogenetic information and more reliably correlated with outcome. In this classification, patients with t(8;21), inv(16), t(15;17), or MLL translocations, which collectively constituted nearly half of the cases of childhood AML, were classified as AML with recurrent cytogenetic abnormalities. This classification system also decreased the bone marrow percentage of leukemic blast requirement for the diagnosis of AML from 30% to 20%; an additional clarification was made so that patients with recurrent cytogenetic abnormalities did not need to meet the minimum blast requirement to be considered AML.[8,9,10]