Name

Acute myeloid leukemia with biallelic mutation of CEBPA

ICD-O-3 Morphology

9878/3: Acute myeloid leukemia with biallelic mutation of CEBPA
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.)

AML with biallelic mutation of CEBPA tends to be associated with higher hemoglobin levels, lower platelet counts, and lower lactate dehydrogenase levels. It may also be associated with a lower frequency of lymphadenopathy.

Grade

Not Applicable

Module Rule

None

Alternate Names

None

Definition

Acute myeloid leukemia (AML) with biallelic mutation of CEBPA usually meets the criteria for AML with maturation or AML without maturation, but some cases show myelomonocytic or monoblastic features. This leukemia usually presents de novo.

Definitive Diagnostic Methods

None

Genetics Data

Biallelic mutation
CEBPA mutation
FLT3-ITD mutation
GATA2 zinc finger 1 mutation

Immunophenotyping

CD7 expression
CD11b expression
CD13 expression
CD15 expression
CD33 expression
CD34 expression
CD56 expression
CD65 expression
HLA-DR expression

Treatments

Chemotherapy

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
Easy bruising or bleeding
Elevated white blood cell count
Fatigue
Fever
Hepatosplenomegaly
Organomegaly
Petechiae
Shortness of breath
Splenomegaly
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: all ages
Incidence: 4-9% of all children and adults with AML
Survival: favorable prognosis

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: 142-144

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

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