Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with RUNX1::RUNX1T1

ICD-O-3 Morphology

9896/3: Acute myeloid leukemia, t(8;21)(q22;q22) RUNX1-RUNX1T1
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 RUNX1::RUNX1T1 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. Some patients may present with myeloid sarcoma.

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, AML1(CBF-Alpha)/ETO (AML1/ETO)
Acute myeloid leukemia, t(8;21)(q22;q22.1); RUNX1-RUNX1T1
Acute myeloid leukemia with t(8;21)(q22;q22.1)
FAB M2, AML1(CBF-Alpha)/ETO
FAB M2, t(8;21)(q22;q22)

Definition

Acute myeloid leukemia (AML) with RUNX1::RUNX1T1 is characterized by fusion of the RUNX family transcription factor 1 gene (RUNX1) and the RUNX1 partner transcriptional co-repressor 1 gene (RUNX1T1). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

RUNX1-RUNX1T1
t(8;21)(q22;q22.1)

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)

C92.0 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 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
Flow cytometry
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

High complete remission rate with long term disease-free survival when treated with high dose cytarabine in the consolidation phase

Epidemiology and Mortality

Age: predominantly younger patients
Incidence: ~5-10% of AML cases
Survival: High complete remission with possible long term disease free survival when treated with high dose cytarabine

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: 115-117

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