Chronic neutrophilic leukemia

Name
ICD-O-1 Morphology
9867/3
Effective 1978 - 1991
ICD-O-2 Morphology
9803/3
Effective 1992 - 2000
ICD-O-3 Morphology
9963/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
CNL
Definition
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 focuses on control rather than cure. The patient is usually treated with hydroxyurea.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Peripheral blood smear
Genetics Data
None
Immunophenotyping
None
Treatments
Blood thinners, anti-coagulant medications, aspirin
Bone marrow transplant
Chemotherapy
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 (effective October 1, 2015 U.S. only)
D47.1 Chronic myeloproliferative disease
Signs and Symptoms
Bleeding from mucocutaneous surfaces
GI bleeding
Gout
Pruritus
Splenomegaly
Diagnostic Exams
Recurrence and Metastases
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