Chronic neutrophilic leukemia: Difference between revisions
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'''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}} | '''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}} | ||
{{AE}}[[Homa Najafi , M.D.]] | |||
==Overview== | ==Overview== | ||
| Line 98: | Line 98: | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
Progression to acute myeloid leukemia may seen in 10-21.2% of patients with CNL.<ref name="Elliott2006">{{cite journal|last1=Elliott|first1=Michelle A.|title=Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined|journal=Best Practice & Research Clinical Haematology|volume=19|issue=3|year=2006|pages=571–593|issn=15216926|doi=10.1016/j.beha.2005.07.012}}</ref><ref name="Reilly2002">{{cite journal|last1=Reilly|first1=John T.|title=CHRONIC NEUTROPHILIC LEUKAEMIA: A DISTINCT CLINICAL ENTITY?|journal=British Journal of Haematology|volume=116|issue=1|year=2002|pages=10–18|issn=0007-1048|doi=10.1046/j.1365-2141.2002.03234.x}}</ref> | Progression to acute myeloid leukemia may seen in 10-21.2% of patients with CNL.<ref name="Elliott2006">{{cite journal|last1=Elliott|first1=Michelle A.|title=Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined|journal=Best Practice & Research Clinical Haematology|volume=19|issue=3|year=2006|pages=571–593|issn=15216926|doi=10.1016/j.beha.2005.07.012}}</ref><ref name="Reilly2002">{{cite journal|last1=Reilly|first1=John T.|title=CHRONIC NEUTROPHILIC LEUKAEMIA: A DISTINCT CLINICAL ENTITY?|journal=British Journal of Haematology|volume=116|issue=1|year=2002|pages=10–18|issn=0007-1048|doi=10.1046/j.1365-2141.2002.03234.x}}</ref> | ||
Prognosis is generally poor, and the 5-year survival rate of patients with CNL is approximately 28%.<ref name="Reilly2002">{{cite journal|last1=Reilly|first1=John T.|title=CHRONIC NEUTROPHILIC LEUKAEMIA: A DISTINCT CLINICAL ENTITY?|journal=British Journal of Haematology|volume=116|issue=1|year=2002|pages=10–18|issn=0007-1048|doi=10.1046/j.1365-2141.2002.03234.x}}</ref><ref>{{Cite journal | Prognosis is generally poor, and the 5-year survival rate of patients with CNL is approximately 28%.<ref name="Reilly2002">{{cite journal|last1=Reilly|first1=John T.|title=CHRONIC NEUTROPHILIC LEUKAEMIA: A DISTINCT CLINICAL ENTITY?|journal=British Journal of Haematology|volume=116|issue=1|year=2002|pages=10–18|issn=0007-1048|doi=10.1046/j.1365-2141.2002.03234.x}}</ref><ref>{{Cite journal | ||
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* Splenomegaly | * Splenomegaly | ||
The common complications of CNL include:<ref name="ElliottHanson2004">{{cite journal|last1=Elliott|first1=M A|last2=Hanson|first2=C A|last3=Dewald|first3=G W|last4=Smoley|first4=S A|last5=Lasho|first5=T L|last6=Tefferi|first6=A|title=WHO-defined chronic neutrophilic leukemia: a long-term analysis of 12 cases and a critical review of the literature|journal=Leukemia|volume=19|issue=2|year=2004|pages=313–317|issn=0887-6924|doi=10.1038/sj.leu.2403562}}</ref><ref name="CigudosaMenezes2015">{{cite journal|last1=Cigudosa|first1=Juan C|last2=Menezes|first2=Juliane|title=Chronic neutrophilic leukemia: a clinical perspective|journal=OncoTargets and Therapy|year=2015|pages=2383|issn=1178-6930|doi=10.2147/OTT.S49688}}</ref> | |||
The | * Predisposing to hemorrhage (intracranial hemorrhage as the most common cause of death in CNL patients) | ||
* Progression of disease | |||
* Blastic or leukemic conversion | |||
* Treatment-related toxicity | |||
The | The indication of disease progression in CNL patients include: | ||
* Treatment-resistant | |||
* Refractory neutrophilia | |||
* Increasing in red blood cells | |||
* Platelet transfusion dependency | |||
* Deterioration of organomegaly | |||
* Blast crisis | |||
==Diagnosis== | ==Diagnosis== | ||
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In the absence of a CSFR3R mutation, persistent neutrophilia (at least 3 months), splenomegaly, and no identifiable cause of reactive neutrophilia | In the absence of a CSFR3R mutation, persistent neutrophilia (at least 3 months), splenomegaly, and no identifiable cause of reactive neutrophilia | ||
including absence of a plasma cell neoplasm or, if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies. | including the absence of a plasma cell neoplasm or, if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies. | ||
|} | |} | ||
Revision as of 19:50, 9 January 2019
For patient information, click here
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Chronic neutrophilic leukemia Microchapters |
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Differentiating Chronic neutrophilic leukemia from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Chronic neutrophilic leukemia On the Web |
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American Roentgen Ray Society Images of Chronic neutrophilic leukemia |
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Directions to Hospitals Treating Chronic neutrophilic leukemia |
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Risk calculators and risk factors for Chronic neutrophilic leukemia |
Associate Editor(s)-in-Chief: Homa Najafi , M.D.
Overview
Chronic neutrophilic leukemia (CNL) is an extremely rare myeloproliferative neoplasms with almost 200 cases in the world. While, most of the time this disease is asymptomatic, fatigue, weight loss, night sweats, bone pain, gout and pruritus are some of its symptoms. Splenomegaly is found in examination of most patients.
Historical Perspective
- Chronic neutrophilic leukemia(CNL) was first presented by Tuohy, in a case of splenomegaly and neutrophilic leukocytosis, in 1920.[1] Although, It was named by Tanzer et al, in 1964.[2]
- In 2001, WHO introduced the criteria for diagnosis CNL as a myeloproliferative disorder that was revised in 2016.[3]
- In 2013, CSF3R (colony stimulating factor 3 receptor) mutations was proposed that was found in the most CNL patients.[4]
Classification
There is no established system for the classification of CNL.
Pathophysiology
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Causes
Disease name] may be caused by [cause1], [cause2], or [cause3].
OR
Common causes of [disease] include [cause1], [cause2], and [cause3].
OR
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
OR
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.
Differentiating ((Page name)) from Other Diseases
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
OR
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
Epidemiology and Demographics
- There are almost only 200 patients with CNL worldwide.[5]
- The exact incidence of CNL is undetermined.[6]
- The incidence of CNL increases with age; the median age at diagnosis is 66.5 years.
- There is no racial predilection to CNL.
- CNL affects men and women almost equally.[7]
Risk Factors
There are no established risk factors for [disease name].
OR
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
Screening
There is insufficient evidence to recommend routine screening for [disease/malignancy].
OR
According to the [guideline name], screening for [disease name] is not recommended.
OR
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
Natural History, Complications, and Prognosis
Progression to acute myeloid leukemia may seen in 10-21.2% of patients with CNL.[7][8]
Prognosis is generally poor, and the 5-year survival rate of patients with CNL is approximately 28%.[8][9]
The factors that can predict poor outcomes are:[10][11][12]
- white blood cell count>50,000 cells per microliter
- ASXL1 mutation
- Thrombocytopenia
- Advanced age
- Dependency to transfusion
- Peripheral blasts ≥ 1%
- Marrow blasts ≥ 10%
- Splenomegaly
The common complications of CNL include:[13][14]
- Predisposing to hemorrhage (intracranial hemorrhage as the most common cause of death in CNL patients)
- Progression of disease
- Blastic or leukemic conversion
- Treatment-related toxicity
The indication of disease progression in CNL patients include:
- Treatment-resistant
- Refractory neutrophilia
- Increasing in red blood cells
- Platelet transfusion dependency
- Deterioration of organomegaly
- Blast crisis
Diagnosis
Diagnostic Study of Choice
The diagnosis of CNL is based on the WHO criteria, which include:[15]
| 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: |
|
| 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:[16][17]
- 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:[13]
- Splenomegaly
- Hepatomegaly
- Lymphadenopathy(uncommon)
Laboratory Findings
A chronic elevated concentration of blood mature neutrophils is diagnostic for CNL.[13]
Some patients with CNL may have:[17][13][7]
- 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:[3][18]
- 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:[13][6][19]
- Hematopoitic stem cell transplant (HSCT)
- Hydroxyurea
- Interferon
- Hypomethylating agents
- Ruxolitinib
- Thalidomide
- Cladribine
- Imatinib
- Splenic irradiation and splenectomy
References
- ↑ Tuohy, E. L. (1920). "A CASE OF SPLENOMEGALY WITH POLYMORPHONUCLEAR NEUTROPHIL HYPERLEUKOCYTOSIS". The American Journal of the Medical Sciences. 160 (1): 18–24. doi:10.1097/00000441-192007000-00003. ISSN 0002-9629.
- ↑ Tanzer, J.; Harel, P.; Boiron, M.; Bernard, Jean (1964). "CYTOCHEMICAL AND CYTOGENETIC FINDINGS IN A CASE OF CHRONIC NEUTROPHILIC LEUKÆMIA OF MATURE CELL TYPE". The Lancet. 283 (7329): 387–388. doi:10.1016/S0140-6736(64)92142-7. ISSN 0140-6736.
- ↑ 3.0 3.1 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.
- ↑ Maxson, Julia E.; Gotlib, Jason; Pollyea, Daniel A.; Fleischman, Angela G.; Agarwal, Anupriya; Eide, Christopher A.; Bottomly, Daniel; Wilmot, Beth; McWeeney, Shannon K.; Tognon, Cristina E.; Pond, J. Blake; Collins, Robert H.; Goueli, Basem; Oh, Stephen T.; Deininger, Michael W.; Chang, Bill H.; Loriaux, Marc M.; Druker, Brian J.; Tyner, Jeffrey W. (2013). "Oncogenic CSF3R Mutations in Chronic Neutrophilic Leukemia and Atypical CML". New England Journal of Medicine. 368 (19): 1781–1790. doi:10.1056/NEJMoa1214514. ISSN 0028-4793.
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ 6.0 6.1 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.
- ↑ 7.0 7.1 7.2 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.
- ↑ 8.0 8.1 Reilly, John T. (2002). "CHRONIC NEUTROPHILIC LEUKAEMIA: A DISTINCT CLINICAL ENTITY?". British Journal of Haematology. 116 (1): 10–18. doi:10.1046/j.1365-2141.2002.03234.x. ISSN 0007-1048.
- ↑ J. Bohm & H. E. Schaefer (2002). "Chronic neutrophilic leukaemia: 14 new cases of an uncommon myeloproliferative disease". Journal of clinical pathology. 55 (11): 862–864. PMID 12401827. Unknown parameter
|month=ignored (help) - ↑ Dao, Kim-Hien T.; Tyner, Jeffrey W.; Gotlib, Jason (2017). "Recent Progress in Chronic Neutrophilic Leukemia and Atypical Chronic Myeloid Leukemia". Current Hematologic Malignancy Reports. 12 (5): 432–441. doi:10.1007/s11899-017-0413-y. ISSN 1558-8211.
- ↑ Elliott, Michelle A.; Pardanani, Animesh; Hanson, Curtis A.; Lasho, Terra L.; Finke, Christy M.; Belachew, Alem A.; Tefferi, Ayalew (2015). "ASXL1mutations are frequent and prognostically detrimental inCSF3R-mutated chronic neutrophilic leukemia". American Journal of Hematology. 90 (7): 653–656. doi:10.1002/ajh.24031. ISSN 0361-8609.
- ↑ Massimo Breccia, Francesca Biondo, Roberto Latagliata, Ida Carmosino, Franco Mandelli & Giuliana Alimena (2006). "Identification of risk factors in atypical chronic myeloid leukemia". Haematologica. 91 (11): 1566–1568. PMID 17043019. Unknown parameter
|month=ignored (help) - ↑ 13.0 13.1 13.2 13.3 13.4 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.
- ↑ Cigudosa, Juan C; Menezes, Juliane (2015). "Chronic neutrophilic leukemia: a clinical perspective". OncoTargets and Therapy: 2383. doi:10.2147/OTT.S49688. ISSN 1178-6930.
- ↑ 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.
- ↑ 17.0 17.1 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.
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ 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.