Acute Myeloid Leukemia With Mutated NPM1

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Home Registry Operations Reporting Guidelines Hematopoietic Project Neoplasm Information Acute myeloid leukemia with NPM1 mutation (see 9861/3 prior to 2021) 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)

Help me code for diagnosis year : --2001-- --2002-- --2003-- --2004-- --2005-- --2006-- --2007-- --2008-- --2009-- --2010-- --2011-- --2012-- --2013-- --2014-- --2015-- --2016-- --2017-- --2018-- --2019-- --2020-- 2021 2022 2023 2024 2025 2026 2027

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

Acute myeloid leukemia with mutated NPM1

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

CytogeneticsGenetic testing

Genetics Data

Mutated NPM1

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

ChemotherapyHematologic Transplant and/or Endocrine Procedures

Transformations to

None

Transformations from

9875/3 Chronic myeloid leukemia (CML), BCR::ABL1 positive 9920/3 Myeloid neoplasm post cytotoxic therapy (MN-pCTs) 9945/3 Chronic myelomonocytic leukemia, NOS (CMML) 9950/3 Polycythemia vera (PV) 9960/3 Myeloproliferative neoplasm, NOS [OBS] (see 9975/3 for 2010+) 9961/3 Primary myelofibrosis, NOS (PMF) 9962/3 Essential thrombocythemia (ET) 9963/3 Chronic neutrophilic leukemia (CNL) 9964/3 Chronic eosinophilic leukemia (CEL) 9975/3 Myelodysplastic/myeloproliferative neoplasm, NOS, unclassifiable (MPN/MDS-U) 9980/3 Myelodysplastic neoplasm with low blasts and single-lineage dysplasia (MDS-LB-SLD) 9982/3 Myelodysplastic /myeloproliferative neoplasm with low blasts and SF3B1 mutation 9983/3 Myelodysplastic neoplasm with increased blasts (MDS-IB) 9984/3 Refractory anemia with excess blasts in transformation (RAEB-T) [OBS] 9985/3 Myelodysplastic neoplasm with low blasts, NOS (MDS-LB) 9986/3 Myelodysplastic neoplasm with low blasts and 5q deletion (MDS-5q) 9987/3 Therapy-related myelodysplastic syndrome (t-MDS), NOS 9989/3 Myelodysplastic neoplasm, NOS 9991/3 Refractory neutropenia (see 9980/3 prior to 2010, see 9980/3 for 2021+) 9992/3 Refractory thrombocytopenia 9993/3 Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD)

Same Primaries

9800/3 Leukemia, NOS 9801/3 Acute undifferentiated leukemia 9860/3 Myeloid leukemia, NOS 9861/3 Acute myeloid leukemia (AML), NOS

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

AnemiaBone marrow failureEasy bruising or bleedingFatigueFeverLeukocytosisNeutropeniaPetechiaeShortness of breathThrombocytopeniaWeaknessWeight loss or loss of appetite

Diagnostic Exams

Bone marrow aspiration and biopsyComplete blood count (CBC)CT (CAT) scanCytogenetic analysisFlow cytometryImmunophenotypingLumbar punctureMolecular analysisPhysical exam and historyPeripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

Age: Occurs in 2-8% of childhood cases and 27%-35% of adults casesIncidence: One of the most common recurrent genetic lesions in AML, female predominanceSurvival: 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 leukemiaPages: 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 CodesPages: 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 VersionPages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq Glossary