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
for cases diagnosed 1978 and later
Primary Site(s)
Primary site must be bone marrow (C421)

9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
Alternate Names
Rare myeloproliferative disease. Sustained peripheral blood neutrophilia, bone marrow hypercellularity due to neutrophilic granulocyte proliferation, and hepatosplenomagaly.
Abstractor Notes
Chronic neutrophilic leukemia (CNL) is very rare.

The peripheral blood smear shows neutrophilia and the bone marrow shows hypercellularity but neither are diagnostic of CNL. This is a diagnosis of exclusion; the physician puts together the equivocal test results with the clinical picture and diagnoses CNL.

The peripheral blood and bone marrow are always involved and the spleen and liver usually show leukemic infiltrate; however any tissue may be infiltrated by the neutrophils.

Treatment of CNL focuses on disease 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)
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Peripheral blood smear
Genetics Data
Bone marrow transplant
Transformations from
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 disease (true incidence unknown)
Sex: no male or female predominance
Survival: 6 months to 20 years