Search Database ICD-O-3 Code Lists

Name

Myelodysplasia-related acute myeloid leukemia (AML-MR)

ICD-O-3 Morphology

9895/3: Acute myeloid leukemia with myelodysplasia-related changes
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Myelodysplasia-related acute myeloid leukemia (AML-MR) 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)

The peripheral blood and bone marrow are always involved. Rarely, AML-MR may manifest as myeloid sarcoma.

On presentation, these patients usually have morphological evidence of multilineage dysplasia. This does not mean the patient had a prior MDS, or a history of prior cytotoxic therapy.

Do not automatically assign this histology if patient has a history of a myeloproliferative neoplasm (Myelodysplastic Syndrome [MDS], or Myeloproliferative Neoplasm [MPN]).

The pathologist must make the diagnosis of being myelodysplasia-related.

If this leukemia 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

Acute myeloid leukemia post myelodysplastic-myeloproliferative neoplasm
Acute myeloid leukemia with multilineage dysplasia
Acute myeloid leukemia with myelodysplasia related changes
Acute myeloid leukemia with prior myelodysplastic neoplasm (syndrome)
Oligoblastic AML-MR

Definition

Myelodysplasia-related acute myeloid leukemia (AML-MR) is a myeloid neoplasm harbouring specific cytogenetic and/or molecular abnormalities associated with myelodysplastic neoplasia, arising de novo or after a known diagnosis of myelodysplastic neoplasm (MDS) or myelodysplastic/myeloproliferative neoplasm (MDS/MPN). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation

Genetics Data

5 q deletion or loss of 5 q due to unbalanced translocation
7q deletion (Monosomy 7), or loss of 7q due to unbalanced translocation
12p deletion or loss of 12p due to unbalanced translocation
13 q deletion (Monosomy 13)
17p deletion or loss of 17p due to unbalanced translocation
idic(X)(q13)
Isochromosome 17q

Immunophenotyping

CD34+ (expression/positive)
HLA-DR- (decreased/no expression/negative)
KIT (CD117)- (decreased/no expression/negative)

Treatments

Chemotherapy

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.A Acute myeloid leukemia with multilineage dysplasia (effective October 01, 2015 - September 30, 2024)
C92.A0 Acute myeloid leukemia with multilineage dysplasia not having achieved remission (effective October 01, 2024)
C92.A1 Acute myeloid leukemia with multilineage dysplasia, in remission (effective October 01, 2024)
C92.A2 Acute myeloid leukemia with multilineage dysplasia, in relapse (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
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

Lower rate of achieving complete remission than other AML types

Epidemiology and Mortality

Age: elderly, rare in children
Incidence: 24-35% of all AML cases (variable based on definition)
Survival: generally poor, lower rate of achieving remission than other types of AML

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: 140-143

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

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
Glossary