Chronic neutrophilic leukemia
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Homa Najafi, M.D.[2]
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating chronic neutrophilic leukemia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
The diagnosis of CNL is based on the WHO criteria, which include:[1]
| World Health Organization (WHO) Criteria for CNL Diagnosis | |
|---|---|
| 1. Peripheral blood White blood cells(WBC) ≥25 × 109/L: |
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| 2. Hypercellular bone marrow: |
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| 3. Not meeting WHO criteria for: |
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| 4.No rearrangement of: |
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| 5.Presence of CSF3RT618I or other activating CSF3R mutation or
In the absence of a CSFR3R mutation, persistent neutrophilia (at least 3 months), splenomegaly, and no identifiable cause of reactive neutrophilia including the absence of a plasma cell neoplasm or, if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies. | |
History and Symptoms
The majority of patients with CNL are asymptomatic.
Common symptoms of CNL patients include following:[2][3]
- Fatigue (as a most common symptom)
- Weight loss
- Night sweats
- Bone pain
- Easy bruising
- Pruritus
- Gout
Physical Examination
Physical examinations of patients with CNL include:[4]
- Splenomegaly
- Hepatomegaly
- Lymphadenopathy(uncommon)
Laboratory Findings
A chronic elevated concentration of blood mature neutrophils is diagnostic for CNL.[4]
Some patients with CNL may have:[3][4][5]
- Mild anemia
- Thrombocytopenia
- Elevation of lactate dehydrogenase (LDH)
- Elevation of vitamin B12
Other Diagnostic Studies
Bone marrow morphology
Bone marrow morphology in CNL patient may show:[6][7]
- Hypercellularity with myeloid hyperplasia
- Increasing myeloid to erythroid ratio
- Increasing of myelocytes, metamyelocytes, and bands
- Absence of basophilia and eosinophilia
- Megakaryocytic hyperplasia
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
There is no established treatment for patients with CNL. However, following options may be useful in treatment of patients with CNL:[4][8][9]
- Hematopoitic stem cell transplant (HSCT)
- Hydroxyurea
- Interferon
- Hypomethylating agents
- Ruxolitinib
- Thalidomide
- Cladribine
- Imatinib
- Splenic irradiation and splenectomy
References
- ↑ Arber, D. A.; Orazi, A.; Hasserjian, R.; Thiele, J.; Borowitz, M. J.; Le Beau, M. M.; Bloomfield, C. D.; Cazzola, M.; Vardiman, J. W. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–2405. doi:10.1182/blood-2016-03-643544. ISSN 0006-4971.
- ↑ Reilly JT (2002). "Chronic neutrophilic leukaemia: a distinct clinical entity?". Br J Haematol. 116 (1): 10–8. PMID 11841395.
- ↑ 3.0 3.1 Hasle, Henrik; Olesen, Gitte; Kerndrup, GITTE; Philip, Preben; Jacobsen, Niels (1996). "Chronic neutrophil leukaemia in adolescence and young adulthood". British Journal of Haematology. 94 (4): 628–630. doi:10.1046/j.1365-2141.1996.7082329.x. ISSN 0007-1048.
- ↑ 4.0 4.1 4.2 4.3 Elliott, M A; Hanson, C A; Dewald, G W; Smoley, S A; Lasho, T L; Tefferi, A (2004). "WHO-defined chronic neutrophilic leukemia: a long-term analysis of 12 cases and a critical review of the literature". Leukemia. 19 (2): 313–317. doi:10.1038/sj.leu.2403562. ISSN 0887-6924.
- ↑ Elliott, Michelle A. (2006). "Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined". Best Practice & Research Clinical Haematology. 19 (3): 571–593. doi:10.1016/j.beha.2005.07.012. ISSN 1521-6926.
- ↑ Uppal, Guldeep; Gong, Jerald (2015). "Chronic neutrophilic leukaemia". Journal of Clinical Pathology. 68 (9): 680–684. doi:10.1136/jclinpath-2015-203060. ISSN 0021-9746.
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ Szuber, Natasha; Tefferi, Ayalew (2018). "Chronic neutrophilic leukemia: new science and new diagnostic criteria". Blood Cancer Journal. 8 (2). doi:10.1038/s41408-018-0049-8. ISSN 2044-5385.
- ↑ You W, Weisbrot IM (1979). "Chronic neutrophilic leukemia. Report of two cases and review of the literature". Am J Clin Pathol. 72 (2): 233–42. PMID 289288.