Search Database ICD-O-3 Code Lists

Name

Chronic neutrophilic leukemia (CNL)

ICD-O-3 Morphology

9963/3: Chronic neutrophilic leukemia
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

Chronic neutrophilic leukemia (CNL) is part of the Myeloproliferative neoplasms (MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B2)

The diagnosis of CNL requires careful exclusion of underlying causes of reactive neutrophilia and other neoplastic disorders such as myeloproliferative neoplasms (MPN) and MDS/MPN.

Peripheral blood and bone marrow are always involved, and the spleen and liver usually show leukemic infiltrates.

Presence of the activating CSF3R mutation is histological confirmation of this disease in most cases.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

None

Definition

"Chronic neutrophilic leukemia (CNL) is a myeloproliferative neoplasm characterized by sustained isolated peripheral blood neutrophilia and the presence of activating CSF3R mutations in most cases, without morphological dysplasia or detectable BCR::ABL1." (WHO 5th edition).

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation

Genetics Data

BCR-ABL1 fusion absent
CSF3Rp.T6181 or another activating CSF3R mutation

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

D47.1 Chronic myeloproliferative disease

Corresponding ICD-10-CM Codes (U.S. only)

D47.1 Chronic myeloproliferative disease (effective October 01, 2015)

Signs and Symptoms

Anemia (mild)
Bleeding from mucocutaneous surfaces
Easy bruising
Fatigue
GI bleeding
Gout
Hepatomegaly
Pruritus
Severe infection
Splenomegaly
Sustained mature peripheral blood neutrophilia

Diagnostic Exams

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

Progression and Transformation

Development of myelodysplastic features may indicate transformation to AML

Epidemiology and Mortality

Age: Median age 66, mostly older adults, reported in adolescence and young adults
Incidence: Rare neoplasm (0.1 cases per 1 million persons)
Male to female ratio: 1:6:1
Survival: 6 months to 20 years

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: Myeloproliferative neoplasms
Pages: Part A: 34-36

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 Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <09/27/24>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Accessed <01/22/25>. [PMID: 26389291]
Section: Myeloproliferative Neoplasms Treatment (PDQ®)-Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment
Glossary