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Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Acute myeloid leukemia (AML), NOS is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)
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 basophilic leukemia (9870/3)
2. Acute erythroid leukemia (9840/3)
3. Acute megakaryoblastic leukemia (9910/3)
4. Acute myeloid leukemia, myelodysplasia-related (9895/3)
5. Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 (9911/3)
6. Acute myeloid leukemia with BCR-ABL1 (9912/3) (2021)+ (See 9861/3 prior to 2021)
7. Acute myeloid leukemia with biallelic mutation of CEBPA (9878/3) (2021+) (See 9861/3 prior to 2021)
8. Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (9869/3)
9. Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 (9871/3)
10. Acute myeloid leukemia with maturation (9874/3)
11. Acute myeloid leukemia with minimal differentiation (9872/3)
12. Acute myeloid leukemia with mutated NPM1 (9877/3) (2021+) (See 9861/3 prior to 2021)
13. Acute myeloid leukemia with mutated RUNX1 (9879/3) (2021+) (See 9861/3 prior to 2021)
14. Acute myeloid leukemia with myelodysplasia-related changes (9895/3)
15. Acute myeloid leukemia with t(6;9)(p23;q34.1); DEK-NUP214 (9865/3)
16. Acute myeloid leukemia, t(8;21)(q22;q22.1); RUNX1-RUNX1T1 (9896/3)
17.Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 (9897/3)
18. Acute myeloid leukemia without maturation (9873/3)
19. Acute myelomonocytic leukemia (9867/3)
20. Acute monocytic leukemia (9891/3)
21. Myeloid leukemia associated with Down syndrome (9898/3)
When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code. See the Lineage Tables in the Hematopoietic manual 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.
Starting in the WHO Blue Book, 5th edition for Hematolymphoid tumors, several new terms, based on genetics were added to 9861/3. These terms can be found below and in the alternate names.
Specific AML histologies with recurrent genetic abnormalities that are coded as 9861/3 include (document in your text the specific leukemia):
9861/3: Acute myeloid leukemia with CBFA2T3:GLIS2 fusion
9861/3: Acute myeloid leukemia with FUS::ERG fusion
9861/3: Acute myeloid leukemia with KAT6A::CREBBP fusion
9861/3: Acute myeloid leukemia with MNX1::ETV6 fusion
9861/3: Acute myeloid leukemia with NPM1::MLF1 fusion
9861/3: Acute myeloid leukemia with NUP98 rearrangement
Other AML histologies with recurrent genetic abnormalities and unique ICD-O-3 codes include:
9865: Acute myeloid leukemia with DEK::NUP214 fusion
9866: Acute promyelocytic leukemia with PML::RARA fusion
9869: Acute myeloid leukemia with MECOM rearrangement
9871: Acute myeloid leukemia with CBFB-MYH11 fusion
9877: Acute myeloid leukemia with mutated NPM1 (2021+) (See 9861/3 prior to 2021)
9878: Acute myeloid leukemia with CEBPA mutation (2021+) (See 9861/3 prior to 2021)
9879: Acute myeloid leukemia with mutated RUNX1 (2021+) (See 9861/3 prior to 2021)
9895/3: Acute myeloid leukemia, myelodysplasia related
9896: Acute myeloid leukemia with RUNX1-RUNX1T1 fusion
9897: Acute myeloid leukemia with KMT2A rearrangement
9911: Acute myeloid leukemia with RBM15::MRTFA fusion
9912: Acute myeloid leukemia with BCR-ABL1 (2021+) (See 9861/3 prior to 2021)
If an AML and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.
If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.
See Multiple Primary Rule M3
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 basophilic leukemia (9870/3)
2. Acute erythroid leukemia (9840/3)
3. Acute megakaryoblastic leukemia (9910/3)
4. Acute myeloid leukemia, myelodysplasia-related (9895/3)
5. Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 (9911/3)
6. Acute myeloid leukemia with BCR-ABL1 (9912/3) (2021)+ (See 9861/3 prior to 2021)
7. Acute myeloid leukemia with biallelic mutation of CEBPA (9878/3) (2021+) (See 9861/3 prior to 2021)
8. Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (9869/3)
9. Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 (9871/3)
10. Acute myeloid leukemia with maturation (9874/3)
11. Acute myeloid leukemia with minimal differentiation (9872/3)
12. Acute myeloid leukemia with mutated NPM1 (9877/3) (2021+) (See 9861/3 prior to 2021)
13. Acute myeloid leukemia with mutated RUNX1 (9879/3) (2021+) (See 9861/3 prior to 2021)
14. Acute myeloid leukemia with myelodysplasia-related changes (9895/3)
15. Acute myeloid leukemia with t(6;9)(p23;q34.1); DEK-NUP214 (9865/3)
16. Acute myeloid leukemia, t(8;21)(q22;q22.1); RUNX1-RUNX1T1 (9896/3)
17.Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 (9897/3)
18. Acute myeloid leukemia without maturation (9873/3)
19. Acute myelomonocytic leukemia (9867/3)
20. Acute monocytic leukemia (9891/3)
21. Myeloid leukemia associated with Down syndrome (9898/3)
When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code. See the Lineage Tables in the Hematopoietic manual 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.
Starting in the WHO Blue Book, 5th edition for Hematolymphoid tumors, several new terms, based on genetics were added to 9861/3. These terms can be found below and in the alternate names.
Specific AML histologies with recurrent genetic abnormalities that are coded as 9861/3 include (document in your text the specific leukemia):
9861/3: Acute myeloid leukemia with CBFA2T3:GLIS2 fusion
9861/3: Acute myeloid leukemia with FUS::ERG fusion
9861/3: Acute myeloid leukemia with KAT6A::CREBBP fusion
9861/3: Acute myeloid leukemia with MNX1::ETV6 fusion
9861/3: Acute myeloid leukemia with NPM1::MLF1 fusion
9861/3: Acute myeloid leukemia with NUP98 rearrangement
Other AML histologies with recurrent genetic abnormalities and unique ICD-O-3 codes include:
9865: Acute myeloid leukemia with DEK::NUP214 fusion
9866: Acute promyelocytic leukemia with PML::RARA fusion
9869: Acute myeloid leukemia with MECOM rearrangement
9871: Acute myeloid leukemia with CBFB-MYH11 fusion
9877: Acute myeloid leukemia with mutated NPM1 (2021+) (See 9861/3 prior to 2021)
9878: Acute myeloid leukemia with CEBPA mutation (2021+) (See 9861/3 prior to 2021)
9879: Acute myeloid leukemia with mutated RUNX1 (2021+) (See 9861/3 prior to 2021)
9895/3: Acute myeloid leukemia, myelodysplasia related
9896: Acute myeloid leukemia with RUNX1-RUNX1T1 fusion
9897: Acute myeloid leukemia with KMT2A rearrangement
9911: Acute myeloid leukemia with RBM15::MRTFA fusion
9912: Acute myeloid leukemia with BCR-ABL1 (2021+) (See 9861/3 prior to 2021)
If an AML and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.
If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.
See Multiple Primary Rule M3
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.
Module Rule
See abstractor notes
Alternate Names
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.
Additional definitions included in 9861/3 based on the WHO Blue Book 5th edition:
1. Acute myeloid leukemia (AML) with NUP98 rearrangement is characterized by chromosomal translocations involving NUP98 on chromosome 11p15.4 and various partner genes.
2. Acute myeloid leukemia (AML) with other defined genetic alterations includes emerging AML subtypes with distinct genetic features (see abstractor notes for complete list)
Additional definitions included in 9861/3 based on the WHO Blue Book 5th edition:
1. Acute myeloid leukemia (AML) with NUP98 rearrangement is characterized by chromosomal translocations involving NUP98 on chromosome 11p15.4 and various partner genes.
2. Acute myeloid leukemia (AML) with other defined genetic alterations includes emerging AML subtypes with distinct genetic features (see abstractor notes for complete list)
Definitive Diagnostic Methods
Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Genetics Data
CBFA2T3::GLIS2 fusion
FLT3-ITD
FUS::ERG fusion
KAT6A::CREBBP fusion
MNX1::ETV6 fusion
NPM1::MLF1 fusion
NUP98 rearrangement
Immunophenotyping
None
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Transformations to
None
Transformations from
Same Primaries
Corresponding ICD-10 Codes (Cause of Death codes only)
C92.0 Acute myeloid leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C92.0 Acute myeloblastic leukemia (effective October 01, 2015 - September 30, 2024)
C92.00 Acute myeloblastic leukemia not having achieved remission (effective October 01, 2024)
C92.01 Acute myeloblastic leukemia, in remission (effective October 01, 2024)
C92.02 Acute myeloblastic leukemia, in remission (effective October 01, 2024)
Signs and Symptoms
Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Flow cytometry
Molecular analysis
Progression and Transformation
None
Epidemiology and Mortality
None
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Acute myeloid leukemia
Pages: Part A: 133-134, 144-146
Section: Acute myeloid leukemia
Pages: Part A: 133-134, 144-146
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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
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
PDQ® Adult Treatment Editorial Board. PDQ Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
