Chronic neutrophilic leukemia: Difference between revisions

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__NOTOC__
__NOTOC__
'''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}}
{{CMG}}; {{AE}} {{Homa}}; {{GRR}} {{Nat}}


'''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}}
{{SK}} [[CNL]]
{{CMG}}; {{AE}} {{Homa}}


==[[Chronic neutrophilic leukemia overview|Overview]]==
==[[Chronic neutrophilic leukemia overview|Overview]]==
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==[[Chronic neutrophilic leukemia classification|Classification]]==
==[[Chronic neutrophilic leukemia classification|Classification]]==


==[[hronic neutrophilic leukemia pathophysiology|Pathophysiology]]==
==[[Chronic neutrophilic leukemia pathophysiology|Pathophysiology]]==


==[[Chronic neutrophilic leukemia causes|Causes]]==
==[[Chronic neutrophilic leukemia causes|Causes]]==
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==[[Chronic neutrophilic leukemia differential diagnosis|Differentiating chronic neutrophilic leukemia from other Diseases]]==
==[[Chronic neutrophilic leukemia differential diagnosis|Differentiating chronic neutrophilic leukemia from other Diseases]]==


==[[hronic neutrophilic leukemia epidemiology and demographics|Epidemiology and Demographics]]==
==[[Chronic neutrophilic leukemia epidemiology and demographics|Epidemiology and Demographics]]==


==[[Chronic neutrophilic leukemia risk factors|Risk Factors]]==
==[[Chronic neutrophilic leukemia risk factors|Risk Factors]]==


==Screening==
==[[Chronic neutrophilic leukemia screening|Screening]]==
There is insufficient evidence to recommend routine screening for CNL.
 
==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>
 
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
| author = [[J. Bohm]] & [[H. E. Schaefer]]
| title = Chronic neutrophilic leukaemia: 14 new cases of an uncommon myeloproliferative disease
| journal = [[Journal of clinical pathology]]
| volume = 55
| issue = 11
| pages = 862–864
| year = 2002
| month = November
| pmid = 12401827
}}</ref>
 
The factors that can predict poor outcomes are:<ref name="DaoTyner2017">{{cite journal|last1=Dao|first1=Kim-Hien T.|last2=Tyner|first2=Jeffrey W.|last3=Gotlib|first3=Jason|title=Recent Progress in Chronic Neutrophilic Leukemia and Atypical Chronic Myeloid Leukemia|journal=Current Hematologic Malignancy Reports|volume=12|issue=5|year=2017|pages=432–441|issn=1558-8211|doi=10.1007/s11899-017-0413-y}}</ref><ref name="ElliottPardanani2015">{{cite journal|last1=Elliott|first1=Michelle A.|last2=Pardanani|first2=Animesh|last3=Hanson|first3=Curtis A.|last4=Lasho|first4=Terra L.|last5=Finke|first5=Christy M.|last6=Belachew|first6=Alem A.|last7=Tefferi|first7=Ayalew|title=ASXL1mutations are frequent and prognostically detrimental inCSF3R-mutated chronic neutrophilic leukemia|journal=American Journal of Hematology|volume=90|issue=7|year=2015|pages=653–656|issn=03618609|doi=10.1002/ajh.24031}}</ref><ref>{{Cite journal
| author = [[Massimo Breccia]], [[Francesca Biondo]], [[Roberto Latagliata]], [[Ida Carmosino]], [[Franco Mandelli]] & [[Giuliana Alimena]]
| title = Identification of risk factors in atypical chronic myeloid leukemia
| journal = [[Haematologica]]
| volume = 91
| issue = 11
| pages = 1566–1568
| year = 2006
| month = November
| pmid = 17043019
}}</ref>
* 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:<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>
* 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:<ref name="DaoTyner2015">{{cite journal|last1=Dao|first1=K.-H. T.|last2=Tyner|first2=J. W.|title=What's different about atypical CML and chronic neutrophilic leukemia?|journal=Hematology|volume=2015|issue=1|year=2015|pages=264–271|issn=1520-4391|doi=10.1182/asheducation-2015.1.264}}</ref>


* Treatment-resistant
==[[Chronic neutrophilic leukemia natural history, compilications and prognosis|Natural History, Complications and Prognosis]]==
* Refractory neutrophilia
* Increasing in red blood cells
* Platelet transfusion dependency
* Deterioration of organomegaly
* Blast crisis


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
[[Chronic neutrophilic leukemia diagnostic study of choice|Diagnostic study of choice]] | [[Chronic neutrophilic leukemia history and symptoms|History and Symptoms]] | [[Chronic neutrophilic leukemia physical examination|Physical Examination]] | [[Chronic neutrophilic leukemia laboratory findings|Laboratory Findings]] | [[Chronic neutrophilic leukemia electrocardiogram|Electrocardiogram]] | [[Chronic neutrophilic leukemia x ray|X-Ray Findings]] | [[Chronic neutrophilic leukemia echocardiography and ultrasound|Ultrasound]] | [[Chronic neutrophilic leukemia CT|CT-Scan Findings]] | [[Chronic neutrophilic leukemia MRI|MRI Findings]] | [[Chronic neutrophilic leukemia other imaging findings|Other Imaging Findings]] | [[Chronic neutrophilic leukemia other diagnostic studies|Other Diagnostic Studies]]
The diagnosis of CNL is based on the WHO criteria, which include:<ref name="ArberOrazi2016">{{cite journal|last1=Arber|first1=D. A.|last2=Orazi|first2=A.|last3=Hasserjian|first3=R.|last4=Thiele|first4=J.|last5=Borowitz|first5=M. J.|last6=Le Beau|first6=M. M.|last7=Bloomfield|first7=C. D.|last8=Cazzola|first8=M.|last9=Vardiman|first9=J. W.|title=The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia|journal=Blood|volume=127|issue=20|year=2016|pages=2391–2405|issn=0006-4971|doi=10.1182/blood-2016-03-643544}}</ref>
 
{| class="wikitable"
|+
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |World Health Organization (WHO) Criteria for CNL Diagnosis
|-
! style="background: #DCDCDC; text-align: center;" |1. Peripheral blood White blood cells(WBC) ≥25 × 109/L:
| style="background: #F5F5F5;" |
* Segmented neutrophils plus band forms ≥80% of WBC
 
* Neutrophil precursors <10% of WBC
 
* Myeloblasts rarely observed
 
* Monocyte count <1 × 109/L
 
* No dysgranulopoies.
|-
! style="background: #DCDCDC; text-align: center;" |2. Hypercellular bone marrow:
| style="background: #F5F5F5;" |
* Neutrophil granulocytes increased in percentage and number
 
* Normal neutrophil maturation
 
* Myeloblasts <5% of nucleated cells
 
|-
! style="background: #DCDCDC; text-align: center;" |3. Not meeting WHO criteria for:
| style="background: #F5F5F5;" |
* BCR-ABL1+ chronic myeloid leukemia,
 
* Polycythemia vera
* Essential thrombocythemia,
* Primary myelofibrosis
|-
! style="background: #DCDCDC; text-align: center;" |4.No rearrangement of:
|
* PDGFRA,
* PDGFRB,
* FGFR1,
* PCM1-JAK2
|-
! colspan="2" |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:<ref name="pmid11841395">{{cite journal| author=Reilly JT| title=Chronic neutrophilic leukaemia: a distinct clinical entity? | journal=Br J Haematol | year= 2002 | volume= 116 | issue= 1 | pages= 10-8 | pmid=11841395 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11841395  }} </ref><ref name="HasleOlesen1996">{{cite journal|last1=Hasle|first1=Henrik|last2=Olesen|first2=Gitte|last3=Kerndrup|first3=GITTE|last4=Philip|first4=Preben|last5=Jacobsen|first5=Niels|title=Chronic neutrophil leukaemia in adolescence and young adulthood|journal=British Journal of Haematology|volume=94|issue=4|year=1996|pages=628–630|issn=0007-1048|doi=10.1046/j.1365-2141.1996.7082329.x}}</ref>
*[[Fatigue (medical)|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:<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>
*Splenomegaly
*Hepatomegaly
*Lymphadenopathy(uncommon)
===Laboratory Findings===
A chronic elevated concentration of blood mature neutrophils is diagnostic for CNL.<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>
 
Some patients with CNL may have:<ref name="HasleOlesen1996">{{cite journal|last1=Hasle|first1=Henrik|last2=Olesen|first2=Gitte|last3=Kerndrup|first3=GITTE|last4=Philip|first4=Preben|last5=Jacobsen|first5=Niels|title=Chronic neutrophil leukaemia in adolescence and young adulthood|journal=British Journal of Haematology|volume=94|issue=4|year=1996|pages=628–630|issn=0007-1048|doi=10.1046/j.1365-2141.1996.7082329.x}}</ref><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="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>
*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:<ref name="UppalGong2015">{{cite journal|last1=Uppal|first1=Guldeep|last2=Gong|first2=Jerald|title=Chronic neutrophilic leukaemia|journal=Journal of Clinical Pathology|volume=68|issue=9|year=2015|pages=680–684|issn=0021-9746|doi=10.1136/jclinpath-2015-203060}}</ref><ref>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>
*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==
==Treatment==
===Medical Therapy===
[[Chronic neutrophilic leukemia medical therapy|Medical Therapy]] | [[Chronic neutrophilic leukemia interventions|Interventions]] | [[Chronic neutrophilic leukemia surgery|Surgery]] | [[Chronic neutrophilic leukemia primary prevention|Primary Prevention]] | [[Chronic neutrophilic leukemia secondary prevention|Secondary Prevention]] | [[Chronic neutrophilic leukemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Chronic neutrophilic leukemia future or investigational therapies|Future or Investigational Therapies]]
There is no established treatment for patients with CNL. However, following options may be useful in treatment of patients with CNL:<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="SzuberTefferi2018">{{cite journal|last1=Szuber|first1=Natasha|last2=Tefferi|first2=Ayalew|title=Chronic neutrophilic leukemia: new science and new diagnostic criteria|journal=Blood Cancer Journal|volume=8|issue=2|year=2018|issn=2044-5385|doi=10.1038/s41408-018-0049-8}}</ref><ref name="pmid289288">{{cite journal| author=You W, Weisbrot IM| title=Chronic neutrophilic leukemia. Report of two cases and review of the literature. | journal=Am J Clin Pathol | year= 1979 | volume= 72 | issue= 2 | pages= 233-42 | pmid=289288 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=289288  }} </ref>
*Hematopoitic stem cell transplant (HSCT)
*Hydroxyurea
*Interferon
*Hypomethylating agents
*Ruxolitinib
*Thalidomide
*Cladribine
*Imatinib
*Splenic irradiation and splenectomy


==References==
==References==
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[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Immunology]]
[[Category:Immunology]]
[[Category:Primary care]]

Latest revision as of 06:32, 28 July 2020

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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]; Grammar Reviewer: Natalie Harpenau, B.S.[3]

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Overview

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