Acute Myeloid Leukemia With Myelodysplasia-related Changes

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Home Registry Operations Reporting Guidelines Hematopoietic Project Neoplasm Information Myelodysplasia-related acute myeloid leukemia (AML-MR) 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)

Help me code for diagnosis year : 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022 2023 2024 2025 2026 2027

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 neoplasmAcute myeloid leukemia with multilineage dysplasiaAcute myeloid leukemia with myelodysplasia related changesAcute myeloid leukemia with prior myelodysplastic neoplasm (syndrome)Acute myeloid leukemia without prior myelodysplastic syndromeOligoblastic 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

CytogeneticsGenetic testingHistologic confirmation

Genetics Data

5 q deletion or loss of 5 q due to unbalanced translocation7q deletion (Monosomy 7), or loss of 7q due to unbalanced translocation11q deletion12p deletion or loss of 12p due to unbalanced translocation13 q deletion (Monosomy 13)17p deletion or loss of 17p due to unbalanced translocationidic(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

9875/3 Chronic myeloid leukemia (CML), BCR::ABL1 positive 9945/3 Chronic myelomonocytic leukemia, NOS (CMML) 9950/3 Polycythemia vera (PV) 9960/3 Myeloproliferative neoplasm, NOS [OBS] (see 9975/3 for 2010+) 9961/3 Primary myelofibrosis, NOS (PMF) 9962/3 Essential thrombocythemia (ET) 9963/3 Chronic neutrophilic leukemia (CNL) 9964/3 Chronic eosinophilic leukemia (CEL) 9975/3 Myelodysplastic/myeloproliferative neoplasm, NOS, unclassifiable (MPN/MDS-U) 9980/3 Myelodysplastic neoplasm with low blasts and single-lineage dysplasia (MDS-LB-SLD) 9982/3 Myelodysplastic /myeloproliferative neoplasm with low blasts and SF3B1 mutation 9983/3 Myelodysplastic neoplasm with increased blasts (MDS-IB) 9984/3 Refractory anemia with excess blasts in transformation (RAEB-T) [OBS] 9985/3 Myelodysplastic neoplasm with low blasts, NOS (MDS-LB) 9986/3 Myelodysplastic neoplasm with low blasts and 5q deletion (MDS-5q) 9987/3 Therapy-related myelodysplastic syndrome (t-MDS), NOS 9989/3 Myelodysplastic neoplasm, NOS 9991/3 Refractory neutropenia (see 9980/3 prior to 2010, see 9980/3 for 2021+) 9992/3 Refractory thrombocytopenia 9993/3 Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD)

Same Primaries

9590/3 Malignant lymphoma, NOS 9800/3 Leukemia, NOS 9801/3 Acute undifferentiated leukemia 9840/3 Acute erythroid leukemia (AEL) 9860/3 Myeloid leukemia, NOS 9861/3 Acute myeloid leukemia (AML), NOS

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 bleedingFatigueFeverPetechiaeSevere pancytopeniaShortness of breathWeaknessWeight loss or loss of appetite

Diagnostic Exams

Bone marrow aspiration and biopsyComplete blood count (CBC)CT (CAT) scanCytogenetic analysisImmunophenotypingLumbar punctureMolecular analysisPeripheral blood smearPhysical exam and history

Progression and Transformation

Lower rate of achieving complete remission than other AML types

Epidemiology and Mortality

Age: elderly, rare in childrenIncidence: 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 leukemiaPages: 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 CodesPages: 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 VersionPages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq Glossary