Chronic neutrophilic leukemia
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| Chronic neutrophilic leukemia Classification and external resources | |
| ICD-9 | 205.1 |
|---|---|
| ICD-O: | 9963/3[1] |
| MeSH | D015467 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-525-6884
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Overview
Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder that features a persistent neutrophilia in peripheral blood, myeloid hyperplasia in bone marrow, hepatosplenomegaly, and the absence of the Philadelphia chromosome or a BCR/ABL fusion gene.[1]
Epidemiology
This is a rare disease, with less than 100 cases reported. Of these cases, an equal male:female ratio was observed,[1] with cases typically seen in older adults.[1]
Clinical Features
Etiology
The etiology 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.[1] However, a clonal genetic abnormality has not been detected in these myeloma-associated cases of CNL, raising the possibility that the neutrophilia is a reaction due to the neoplastic myeloma cells.[1] The postulated cell of origin is a limited-potential, marrow-derived stem cell.[1]
Clinical Presentation
The most common clincal finding is hepatosplenomegaly. Pruritus, gout, and mucocutaneous bleeding are occasionally seen.[1][1]
Laboratory Findings
Peripheral blood neutrophilia (> 25 x 109/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes) comprising less than 5% of leukocytes.[1] [1]
Sites of Involvement
Peripheral blood, bone marrow, spleen, and liver are most common, but any organ or tissue can be infiltrated by neutrophils.[1] [1]
Morphology
Bone Marrow Biopsy
On both the bone marrow aspirate and the core biopsy, a hypercellular marrow with an increased myeloid:erythroid ratio of 20:1 or greater. Myelocytes and neutrophils are increased, and blasts and promyelocytes are not increased. Due to the myeloproliferative nature of the disease, an increase in megakaryocytes and erythroid precursors may be observed, but dyspoiesis in not seen in any cell lineage. Also, reticulin fibrosis is rare.[1][1] 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.[1]
Spleen
Splenic infiltrates are typically found only in the red pulp.[1] [1]
Liver
Hepatic infiltrates can be found in either the sinusoids, portal triad regions, or both.[1] [1]
Molecular Findings
Immunophenotype
No distinct immunophenotype abnormality for CNL has been described.[1]
Genetics
The majority (90%) of cases have not had detectable cytogenetic abnormalities. Most importantly, the Philadelphia chromosome and other BCR/ABL fusion genes are not detected.[1]
Current Articles
References
WikiDoc Research Resources for Chronic neutrophilic leukemia | |
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| Articles on Chronic neutrophilic leukemia | Most recent articles on Chronic neutrophilic leukemia • Most cited articles on Chronic neutrophilic leukemia • Review articles on Chronic neutrophilic leukemia • Articles on Chronic neutrophilic leukemia in N Eng J Med, Lancet, BMJ |
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| Evidence Based Medicine Regarding Chronic neutrophilic leukemia | Cochrane Collaboration on Chronic neutrophilic leukemia • Bandolier on Chronic neutrophilic leukemia • TRIP on Chronic neutrophilic leukemia |
| Cost Effectiveness of Chronic neutrophilic leukemia | Cost Effectiveness of Chronic neutrophilic leukemia |
| Clinical Trials Involving Chronic neutrophilic leukemia | Ongoing Trials on Chronic neutrophilic leukemia at Clinical Trials.gov • Trial results on Chronic neutrophilic leukemia • Clinical Trials on Chronic neutrophilic leukemia at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Chronic neutrophilic leukemia | US National Guidelines Clearinghouse on Chronic neutrophilic leukemia • NICE Guidance on Chronic neutrophilic leukemia • NHS PRODIGY Guidance • FDA on Chronic neutrophilic leukemia • CDC on Chronic neutrophilic leukemia |
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| Genetics, Pharmacogenomics, and Proteinomics of Chronic neutrophilic leukemia | Genetics of Chronic neutrophilic leukemia • Pharmacogenomics of Chronic neutrophilic leukemia • Proteomics of Chronic neutrophilic leukemia |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
