Name

Acute myeloid leukemia, t(8;21)(q22;q22.1); 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 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, AML1(CBF-Alpha)/ETO
Acute myeloid leukemia, FLT3-TKD
Acute myeloid leukemia with recurrent genetic abnormalities
Acute myeloid leukemia with t(8;21)(q22;q22)
Acute myeloid leukemia with t(8;21)(q22;q22); RUNX1-RUNX1T1
AML1/ETO
FAB M2, AML1(CBF-Alpha)/ETO
FAB M2, t(8;21)(q22;q22)

Definition

Acute myeloid leukemia, t(8;21)(q22;q22.1) resulting in RUNX1-RUNX1T1 is an AML showing predominantly neutrophilic maturation. The bone marrow and peripheral blood show large myeloblasts with abundant basophilic cytoplasm, often containing azurophilic granules.

Abstractor Notes

Tumor manifestations such as myeloid sarcomas may be present at diagnosis; in these cases an initial BM aspiration may show a misleading low number of blast cells but should be diagnosed as AML despite a blast percentage in BM of less than 20. Smaller blasts may be found in the PB.

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

ASXL1 and ASXL2 mutations
CBFA
del(9q) with loss of 9q22
KRAS and NRAS cooperating mutation
RUNX1 (also called AML1 and CBFA)
t(8;21)(q22;q22)

Immunophenotyping

CD13 high-intensity expression
CD15 expression
C19 expression
CD33 weak expression
CD34 high-intensity expression
CD56 expression in cases with KIT mutation
CD65 expression
CD79a expression
HLA-DR high-intensity expression
MPO
PAX5 expression
TdT weak expression

Treatments

Chemotherapy

Transformations to

None

Corresponding ICD-9 Codes

205.0 Acute myeloid leukemia

Corresponding ICD-10 Codes

C92.0 Acute myeloid leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C92.0 Acute myeloblastic leukemia (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Shortness of breath
Weakness
Weight loss or loss of appetite

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

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 130-132

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary