HAEM4:Acute Myeloid Leukemia (AML), Not Otherwise Specified

Primary Author(s)*

M. Anwar Iqbal, Ph.D., FFACMG

General Disease Overview / Description of Cancer Category

The category "AML Not Otherwise Specified" or "AML-NOS", as described in the revised 4th edition of the WHO[1], includes eight specific subtypes based on examination of bone marrow aspirates and trephine biopsies as well as peripheral blood smears: AML with Minimal Differentiation, AML with Maturation, AML without Maturation, Acute Myelomonocytic Leukemia, Acute Monoblastic and Monocytic Leukemia, Pure Erythroid Leukemia, Acute Megakaryoblastic Leukemia (AMKL), Acute Basophilic Leukemia, and Acute Panmyelosis with Myelofibrosis. Links to these subtypes are listed below in the "WHO Classification Pages" section. The subtypes in the AML-NOS category do not meet the criteria for inclusion in any of the other AML categories (i.e. AML with Recurrent Genetic Abnormalities, AML with Myelodysplasia-Related Changes, or Therapy-Related Myeloid Neoplasms)[1]. The 2016 revised WHO classification removed from AML-NOS the subcategory Acute Erythroid Leukemia, Erythroid/Myeloid Type[2].


AML-NOS is a diagnosis of exclusion, requiring mutation and cytogenetic analyses to identify prognostically significant genetic alterations which appear to be independent of the morphologic subtypes[1]. If AML with NPM1 and CEPBA are ruled out, the subgroups of AML-NOS are not prognostically significant, with the potential exception of [Pure Erythroid Leukemia[1]. No chromosomal abnormalities have been identified to define AML-NOS specifically; inclusion in the AML-NOS category is primary due to examination of the leukemic cells for morphological and immunophenotypic features[1]. Evaluation of mutational status beyond NPM1 and CEPBA is also important, as reflected by a recent study that found 15% of AML-NOS cases have a RUNX1 mutation which is associated with an intermediate risk[3].

WHO Classification Pages (Includes Links to Content)

  1. HAEM5:Acute myeloid leukaemia with minimal differentiation
  2. HAEM5:Acute myeloid leukaemia without maturation
  3. HAEM5:Acute myeloid leukaemia with maturation
  4. HAEM5:Acute myelomonocytic leukaemia
  5. HAEM5:Acute monocytic leukaemia
  6. HAEM5:Acute erythroid leukaemia
  7. HAEM5:Acute megakaryoblastic leukaemia
  8. HAEM5:Acute basophilic leukaemia
  9. HAEM4:Acute Panmyelosis with Myelofibrosis

Other Related Pages (Includes Links to Content)

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Additional Information

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References

  1. 1.0 1.1 1.2 1.3 1.4 Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p156-166.
  2. Arber, Daniel A.; et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–2405. doi:10.1182/blood-2016-03-643544. ISSN 1528-0020. PMID 27069254.
  3. You, Eunkyoung; et al. (2017). "Frequency and Clinicopathologic Features of RUNX1 Mutations in Patients With Acute Myeloid Leukemia Not Otherwise Specified". American Journal of Clinical Pathology. 148 (1): 64–72. doi:10.1093/ajcp/aqx046. ISSN 1943-7722. PMID 28927163.

Notes

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*The hierarchical tumour classification structure displayed on this page is reproduced from the WHO Classification of Tumours with permission from the copyright holder, ©International Agency for Research on Cancer.