Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with KMT2a rearrangement

ICD-O-3 Morphology

9897/3: Acute myeloid leukemia with t(9;11)(p22;q23);MLLT3-MLL
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

Acute myeloid leukemia with KMT2a rearrangement 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 blood and bone marrow are always involved. Extramedullary involvement is relatively frequent and identified in one third of adult patients with AML with KMT2A rearrangement. Also in children, extramedullary involvement is common, with approximately half of patients presenting with liver and/or splenic involvement, and 15% presenting with CNS involvement

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 AML is part of the "AML with recurrent genetic abnormalities" group. Since this AML is diagnosed based on genetics, diagnostic confirmation will always be 3.

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, MLL
Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL
Acute myeloid leukemia with t(9;11)(p22;q23); or other exact balanced translocation
Acute myeloid leukemia with t(9;11)(p21.3;q23.3) with KMT2A-MLLT3 or other exact fusion
Acute myeloid leukemia with 11q23 (MLL) abnormalities

Definition

Acute myeloid leukemia (AML) with KMT2A rearrangement is a myeloid neoplasm in which the KMT2A locus on chromosome 11q23.3 is fused with any of a number of partner genes. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

KMT2A (previously called MLL and HRX)
KMT2A-MLLT3
MECOM (also called EV11) overexpression
t(9;11)(p21.3;q23.3) involving MLLT3
t(9;11)(p21.3;q23.3) with gain of chromosome 8

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

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)

C92.6 Acute myeloid leukemia with 11q23-abnormality (effective October 01, 2015 - September 30, 2024)
C92.60 Acute myeloid leukemia with 11q23-abnormality not having achieved remission (effective October 01, 2024)
C92.61 Acute myeloid leukemia with 11q23-abnormality, in remission (effective October 01, 2024)
C92.62 Acute myeloid leukemia with 11q23-abnormality, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Dissminated intravascular coagulation
Easy bruising or bleeding
Fatigue
Fever
Neutropenia
Petechiae
Shortness of breath
Thrombocytopenia
Tissue infiltration (gingiva, skin)
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

Progression and Transformation

None

Epidemiology and Mortality

Age: more common in children, occurs also in adults
Incidence: 9-12% of childhood and 2% of adult AML cases
Survival: intermediate survival

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: 127-129

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
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