Name

Acute myeloid leukemia, NOS

ICD-O-1 Morphology

9861/3: Acute myeloid leukemia, NOS
Effective 1978 - 1991

ICD-O-2 Morphology

9861/3: Acute myeloid leukemia, NOS
Effective 1992 - 2000

ICD-O-3 Morphology

9861/3: Acute myeloid leukemia, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Abstractor Notes

Acute myeloid leukemia, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification.

In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis. If no information is found in the medical record, follow-back to the attending physician should be done.

More specific myeloid leukemias include
1. Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 (9911/3)
2. Acute myeloid leukemia with BCR-ABL1 (9912/3) (2021)+
3. Acute myeloid leukemia with biallelic mutation of CEBPA (9878/3) (2021+)
4. Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (9869/3)
5. Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 (9871/3)
6. Acute myeloid leukemia with maturation (9874/3)
7. Acute myeloid leukemia with minimal differentiation (9872/3)
8. Acute myeloid leukemia with mutated NPM1 (9877/3) (2021+)
9. Acute myeloid leukemia with mutated RUNX1 (9879/3) (2021+)
10. Acute myeloid leukemia with myelodysplasia-related changes (9895/3)
11. Acute myeloid leukemia with t(6;9)(p23;q34.1); DEK-NUP214 (9865/3)
12. Acute myeloid leukemia, t(8;21)(q22;q22.1); RUNX1-RUNX1T1 (9896/3)
13.Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 (9897/3)
14. Acute myeloid leukemia without maturation (9873/3)
15. Myeloid leukemia associated with Down syndrome (9898/3)
16. Myeloid/lymphoid neoplasms with PDGFRB rearrangement (9966/3)

When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code. See the histology tables for more information on NOS and more specific histologies.

This histology should also be used when the diagnosis is "Acute myeloid leukemia with recurrent genetic abnormalities" and there are no specific recurrent genetic abnormalities listed.

Specific AML histologies with recurrent genetic abnormalities include:

9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214
9866: Acute promyelocytic leukemia with PML-RARA
9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM
9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11
9877: Acute myeloid leukemia with mutated NPM1 (2021+)
9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+)
9879: Acute myeloid leukemia with mutated RUNX1 (2021+)
9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1
9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3
9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1
9912: Acute myeloid leukemia with BCR-ABL1 (2021)+

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10.

For more information on acute myeloid leukemia, see the NCI website: http://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq or http://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdqAcute myeloid leukemia, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Grade

Not Applicable

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, NOS
Acute myeloid leukemia, NOS (FAB or WHO type not specified)
Acute myeloid leukemia with cytoplasmic nucleophosmin (NPMc + AML)
Acute myeloid leukemia with recurrent genetic abnormalities, NOS
Acute non-lymphocytic leukemia
AML

Definition

Acute myeloid leukemia (AML), NOS, encompasses the cases that do not fulfill the criteria for inclusion in one of the more specified AML entities. The defining criterion for AML is the presence of greater than or equal to 20% myeloid blasts in the peripheral blood or bone marrow.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

FLT3-ITD

Immunophenotyping

CD11b
CD13
CD14
CD33 MPO
CD34
CD68
Varies by subclassification

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Same Primaries

Corresponding ICD-9 Codes

205.0 Acute myeloid leukemia

Corresponding ICD-10 Codes

C92.0 Acute myeloid leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C92.0 Acute myeloblastic leukemia (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

Diagnostic Exams

CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 156-157

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary