Chronic neutrophilic leukemia

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Chronic neutrophilic leukemia
Other namesCNL[1]
SpecialtyHematology and oncology

Chronic neutrophilic leukemia (CNL) is a rare

BCR/ABL fusion gene.[2]

Signs and symptoms

The most common clinical finding is

Pruritus, gout, and mucocutaneous bleeding are occasionally seen.[3][4]

Cause

The cause of CNL is currently unknown. An association between CNL and multiple myeloma has been suggested based on the observation of myeloma in 20% of CNL cases.[5] However, a clonal genetic abnormality has not been detected in these myeloma-associated cases of CNL, raising the possibility that the

myeloma cells.[2] The postulated cell of origin is a limited-potential, marrow-derived stem cell.[6]

Genetics

The majority (90%) of cases have not had detectable

BCR/ABL fusion genes are not detected.[2]

Diagnosis

Laboratory findings

Peripheral blood neutrophilia (> 25 x 109/L) with

Sites of involvement

Peripheral

Bone marrow biopsy

On both the bone marrow aspirate and the core biopsy, a hypercellular marrow with an increased myeloid:

reticulin fibrosis is rare.[3][4] There is a reported association between CNL and multiple myeloma, so the bone marrow biopsy may show evidence of a plasma cell dyscrasia with increased numbers of atypical plasma cells.[2]

Spleen

Splenic infiltrates are typically found only in the red pulp.[3][4]

Liver

portal triad regions, or both.[3][4]

Immunophenotype

No distinct immunophenotype abnormality for CNL has been described.[2] See OHSU 2013 findings of gene CSF3R, mutation p. T6181.

Epidemiology

This is a rare disease, with less than 100 cases reported. Of these cases, an equal male:female ratio was observed,[3] with cases typically seen in older adults.[4]

References

  1. ^ "Chronic neutrophilic leukemia | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 17 November 2019. Retrieved 17 November 2019.
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External links