Name

Acute myeloid leukemia with mutated RUNX1

ICD-O-3 Morphology

9879/3: Acute myeloid leukemia with mutated RUNX1
Effective 2021 and later

Reportable

for cases diagnosed 2021 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9861/3.)

Patients usually present with lower hemoglobin and lactate dehydrogenase levels and lower white blood cell and peripheral blood blast cell counts.

Cases with a history of prior therapy (in particular radiation therapy), MDS, or a myelodysplastic/myeloproliferative neoplasm may harbor RUNX1 mutations, but are excluded from this category.

Grade

Not Applicable

Module Rule

None

Alternate Names

None

Definition

Acute myeloid leukemia (AML) with mutated RUNX1 is a de novo leukemia with greater than or equal to 20% bone marrow or peripheral blood blasts cells that may have morphological features of most AML, NOS categories and has a higher frequency among cases with minimal differentiation.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Karyotyping

Genetics Data

ASXL1
FLT3-ITD
IDH1R132
Karyotypic abnormalities, most commonly trisomies 8 and 13
KMT2A
RUNX1 mutations

Immunophenotyping

CD13 expression
CD33 expression
CD34 expression
HLA-DR expression
MPO expression

Treatments

Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Corresponding ICD-9 Codes

None

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

Anemias
Bone marrow failure
Easy bruising or bleeding
Fatigue
Fever
Leukocytosis
Neutropenia
Petechiae
Shortness of breath
Thrombocytopenia
Weakness
Weight loss or loss of appetite

Diagnostic Exams

CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

Age: Higher frequence in older adults (>60 years)
Incidence: 4-16% of AML cases
Survival: Worse overall survival

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: 144-145

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

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
Glossary