Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with CEBPA mutation (see 9861/3 prior to 2021)

ICD-O-3 Morphology

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

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

Acute myeloid leukemia with CEBPA mutation 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)

Blood and bone marrow are always involved.

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

None

Alternate Names

Acute myeloid leukemia with biallelic mutation of CEBPA (biCEBPA)

Definition

Acute myeloid leukemia (AML) with CEBPA mutation is characterized by CEBPA mutation that should be biallelic (biCEBPA) or, if single, must be located in the bZIP region of the gene (smbZIP-CEBPA). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

biCEBPA
CEBPA mutation
smbZIP-CEBPA

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 remission (effective October 01, 2024)

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

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

None

Epidemiology and Mortality

Age: all ages
Incidence: 4-9% of all children and adults with AML
Survival: favorable prognosis

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: 138-140

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