Name

Chronic neutrophilic leukemia

ICD-O-1 Morphology

Effective 1978 - 1991

ICD-O-2 Morphology

9803/3: Chronic leukemia, NOS
Effective 1992 - 2000

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)

Grade

Not Applicable

Module Rule

None

Alternate Names

CNL

Definition

Chronic neutrophilic leukemia is a rare myeloproliferative neoplasm characterized by sustained peripheral blood neutrophilia, bone marrow hypercellularity due to neutrophilic granulocyte proliferation, and hepatosplenomegaly.

There is no Philadelphia (Ph) chromosome or BCR-ABL2 fusion gene. The diagnoses requires exclusion of reactive neutrophilia and other myeloproliferative and myelodysplastic/myeloproliferative neoplasms.

Abstractor Notes

Treatment of CNL focuses on control rather than cure. Allogeneic bone marrow transplantation represents a potentially curative treatment modality in the management of this disorder. Varying success has been reported with the use of traditional chemotherapies including hydroxyurea and interferon.

Aspirin was previously documented as treatment for this disease. This was found to be incorrect. Treatment has been updated based on the NCI website (updated 6/12/15)

For more information, see the NCI website: https://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_95

Definitive Diagnostic Methods

Bone marrow biopsy
Clinical diagnosis
Peripheral blood smear

Genetics Data

BCR-ABL1 fusion absent
CSF3R mutation (often with SETBP1 or ASXL1)
del(7q)
del(11q)
del(12p)
del(20q) (most frequently observed abnormality)
Gains of chromosomes 8, 9, and 21
Nullisomy 17
Ph chromosome absent

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations from

None

Corresponding ICD-9 Codes

205.1 Chronic myeloid leukemia

Corresponding ICD-10 Codes

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

Bleeding from mucocutaneous surfaces
GI bleeding

Progression and Transformation

Development of myelodysplastic features may indicate transformation to AML

Epidemiology and Mortality

Age: mostly older adults, reported in adolescence
Incidence: Rare neoplasm (true incidence unknown)
Sex: no male or female predominance
Survival: 6 months to 20 years

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: Myeloproliferative neoplasms
Pages: 37-38

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
Glossary