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| __NOTOC__ | | __NOTOC__ |
| | '''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}} |
| | {{CMG}}; {{AE}} {{Homa}}; {{GRR}} {{Nat}} |
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| '''For patient information, click [[Leukemia (patient information)|here]]'''{{Chronic neutrophilic leukemia}}
| | {{SK}} [[CNL]] |
| {{CMG}}; {{AE}} {{Homa}}
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| ==[[Chronic neutrophilic leukemia overview|Overview]]== | | ==[[Chronic neutrophilic leukemia overview|Overview]]== |
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| ==[[Chronic neutrophilic leukemia classification|Classification]]== | | ==[[Chronic neutrophilic leukemia classification|Classification]]== |
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| ==[[hronic neutrophilic leukemia pathophysiology|Pathophysiology]]== | | ==[[Chronic neutrophilic leukemia pathophysiology|Pathophysiology]]== |
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| ==[[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]]== |
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| ==Epidemiology and Demographics== | | ==[[Chronic neutrophilic leukemia epidemiology and demographics|Epidemiology and Demographics]]== |
| * There are almost only 200 patients with CNL worldwide.<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>
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| * The exact incidence of CNL is undetermined.<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>
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| *The incidence of CNL increases with age; the median age at diagnosis is 66.5 years.
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| *There is no racial predilection to CNL.
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| *CNL affects men and women almost equally.<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>
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| ==Risk Factors==
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| There are no established risk factors for CNL.
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| ==Screening==
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| There is insufficient evidence to recommend routine screening for CNL.
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| ==Natural History, Complications, and Prognosis==
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| 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>
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| 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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| | author = [[J. Bohm]] & [[H. E. Schaefer]]
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| | title = Chronic neutrophilic leukaemia: 14 new cases of an uncommon myeloproliferative disease
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| | journal = [[Journal of clinical pathology]]
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| | volume = 55
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| | issue = 11
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| | pages = 862–864
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| | year = 2002
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| | month = November
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| | pmid = 12401827
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| }}</ref>
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| 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
| | ==[[Chronic neutrophilic leukemia risk factors|Risk Factors]]== |
| | author = [[Massimo Breccia]], [[Francesca Biondo]], [[Roberto Latagliata]], [[Ida Carmosino]], [[Franco Mandelli]] & [[Giuliana Alimena]]
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| | title = Identification of risk factors in atypical chronic myeloid leukemia
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| | journal = [[Haematologica]]
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| | volume = 91
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| | issue = 11
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| | pages = 1566–1568
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| | year = 2006
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| | month = November
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| | pmid = 17043019
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| }}</ref>
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| * white blood cell count>50,000 cells per microliter
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| * ASXL1 mutation
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| * Thrombocytopenia
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| * Advanced age
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| * Dependency to transfusion
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| * Peripheral blasts ≥ 1%
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| * Marrow blasts ≥ 10%
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| * Splenomegaly
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| 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>
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| * Predisposing to hemorrhage (intracranial hemorrhage as the most common cause of death in CNL patients)
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| * Progression of disease
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| * Blastic or leukemic conversion
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| * Treatment-related toxicity
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| 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>
| | ==[[Chronic neutrophilic leukemia screening|Screening]]== |
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| * Treatment-resistant
| | ==[[Chronic neutrophilic leukemia natural history, compilications and prognosis|Natural History, Complications and Prognosis]]== |
| * Refractory neutrophilia
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| * Increasing in red blood cells
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| * Platelet transfusion dependency
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| * Deterioration of organomegaly
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| * Blast crisis
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| ==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>
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| {| class="wikitable"
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| ! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |World Health Organization (WHO) Criteria for CNL Diagnosis
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| |-
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| ! style="background: #DCDCDC; text-align: center;" |1. Peripheral blood White blood cells(WBC) ≥25 × 109/L:
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| | style="background: #F5F5F5;" |
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| * Segmented neutrophils plus band forms ≥80% of WBC
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| * Neutrophil precursors <10% of WBC
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| * Myeloblasts rarely observed
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| * Monocyte count <1 × 109/L
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| * No dysgranulopoies.
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| |- | |
| ! style="background: #DCDCDC; text-align: center;" |2. Hypercellular bone marrow:
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| * Neutrophil granulocytes increased in percentage and number
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| * Normal neutrophil maturation
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| * Myeloblasts <5% of nucleated cells
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| |- | |
| ! style="background: #DCDCDC; text-align: center;" |3. Not meeting WHO criteria for:
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| * BCR-ABL1+ chronic myeloid leukemia,
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| * Polycythemia vera
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| * Essential thrombocythemia,
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| * Primary myelofibrosis
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| |-
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| ! style="background: #DCDCDC; text-align: center;" |4.No rearrangement of:
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| * PDGFRA,
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| * PDGFRB,
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| * FGFR1,
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| * PCM1-JAK2
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| |-
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| ! colspan="2" |5.Presence of CSF3RT618I or other activating CSF3R mutation or
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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
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| including the absence of a plasma cell neoplasm or, if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies.
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| |} | |
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| ===History and Symptoms===
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| The majority of patients with CNL are asymptomatic.
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| 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>
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| *[[Fatigue (medical)|Fatigue]] (as a most common symptom)
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| *Weight loss
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| *Night sweats
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| *Bone pain
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| *Easy bruising
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| *Pruritus
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| *Gout
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| ===Physical Examination===
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| 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>
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| *Splenomegaly
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| *Hepatomegaly
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| *Lymphadenopathy(uncommon)
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| ===Laboratory Findings===
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| 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>
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| 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>
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| *Mild anemia
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| *Thrombocytopenia
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| *Elevation of lactate dehydrogenase (LDH)
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| *Elevation of vitamin B12
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| ===Other Diagnostic Studies===
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| ===Bone marrow morphology===
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| 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>
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| *Hypercellularity with myeloid hyperplasia
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| *Increasing myeloid to erythroid ratio
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| *Increasing of myelocytes, metamyelocytes, and bands
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| *Absence of basophilia and eosinophilia
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| *Megakaryocytic hyperplasia
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| [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]. | |
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| OR
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| 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]. | |
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| ==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>
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| *Hematopoitic stem cell transplant (HSCT)
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| *Hydroxyurea
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| *Interferon
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| *Hypomethylating agents
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| *Ruxolitinib
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| *Thalidomide
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| *Cladribine
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| *Imatinib
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| *Splenic irradiation and splenectomy
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| ==References== | | ==References== |
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| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Primary care]]
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