Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with NPM1 mutation (see 9861/3 prior to 2021)

ICD-O-3 Morphology

9877/3: Acute myeloid leukemia with mutated NPM1
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be C421 (bone marrow)

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 NPM1 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)

The blood and bone marrow are nearly always involved. Some patients may present initially with myeloid sarcoma involving the skin, gingiva, lymph nodes, or other extramedullary sites.

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

Definition

Acute myeloid leukemia (AML) with NPM1 mutation is a myeloid neoplasm characterized by the presence of somatic mutations involving the nucleophosmin 1 gene (NPM1). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

Mutated NPM1

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

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

Anemia
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

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

Progression and Transformation

None

Epidemiology and Mortality

Age: Occurs in 2-8% of childhood cases and 27%-35% of adults cases
Incidence: One of the most common recurrent genetic lesions in AML, female predominance
Survival: Good response to induction therapy

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: 135-137

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