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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}} |
| {{CMG}} {{AE}}[[Homa Najafi , M.D.]] | | {{CMG}}; {{AE}} {{Homa}}; {{GRR}} {{Nat}} |
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| ==Overview==
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| 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.
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| ==Historical Perspective==
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| * Chronic neutrophilic leukemia(CNL) was first presented by Tuohy, in a case of splenomegaly and neutrophilic leukocytosis, in 1920.<ref name="Tuohy1920">{{cite journal|last1=Tuohy|first1=E. L.|title=A CASE OF SPLENOMEGALY WITH POLYMORPHONUCLEAR NEUTROPHIL HYPERLEUKOCYTOSIS|journal=The American Journal of the Medical Sciences|volume=160|issue=1|year=1920|pages=18–24|issn=0002-9629|doi=10.1097/00000441-192007000-00003}}</ref> Although, It was named by Tanzer et al, in 1964.<ref name="TanzerHarel1964">{{cite journal|last1=Tanzer|first1=J.|last2=Harel|first2=P.|last3=Boiron|first3=M.|last4=Bernard|first4=Jean|title=CYTOCHEMICAL AND CYTOGENETIC FINDINGS IN A CASE OF CHRONIC NEUTROPHILIC LEUKÆMIA OF MATURE CELL TYPE|journal=The Lancet|volume=283|issue=7329|year=1964|pages=387–388|issn=01406736|doi=10.1016/S0140-6736(64)92142-7}}</ref>
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| * In 2001, WHO introduced the criteria for diagnosis CNL as a myeloproliferative disorder that was revised in 2016.<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>
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| * In 2013, CSF3R (colony stimulating factor 3 receptor) mutations was proposed that was found in the most CNL patients.<ref name="MaxsonGotlib2013">{{cite journal|last1=Maxson|first1=Julia E.|last2=Gotlib|first2=Jason|last3=Pollyea|first3=Daniel A.|last4=Fleischman|first4=Angela G.|last5=Agarwal|first5=Anupriya|last6=Eide|first6=Christopher A.|last7=Bottomly|first7=Daniel|last8=Wilmot|first8=Beth|last9=McWeeney|first9=Shannon K.|last10=Tognon|first10=Cristina E.|last11=Pond|first11=J. Blake|last12=Collins|first12=Robert H.|last13=Goueli|first13=Basem|last14=Oh|first14=Stephen T.|last15=Deininger|first15=Michael W.|last16=Chang|first16=Bill H.|last17=Loriaux|first17=Marc M.|last18=Druker|first18=Brian J.|last19=Tyner|first19=Jeffrey W.|title=Oncogenic CSF3R Mutations in Chronic Neutrophilic Leukemia and Atypical CML|journal=New England Journal of Medicine|volume=368|issue=19|year=2013|pages=1781–1790|issn=0028-4793|doi=10.1056/NEJMoa1214514}}</ref>
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| ==Classification==
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| There is no established system for the classification of CNL.
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| ==Pathophysiology==
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| The exact pathogenesis of [disease name] is not fully understood.
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| 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].
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| [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
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| Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
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| [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
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| The progression to [disease name] usually involves the [molecular pathway].
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| The pathophysiology of [disease/malignancy] depends on the histological subtype.
| | {{SK}} [[CNL]] |
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| ==Causes== | | ==[[Chronic neutrophilic leukemia overview|Overview]]== |
| Disease name] may be caused by [cause1], [cause2], or [cause3].
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| | ==[[Chronic neutrophilic leukemia historical perspective|Historical Perspective]]== |
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| Common causes of [disease] include [cause1], [cause2], and [cause3].
| | ==[[Chronic neutrophilic leukemia classification|Classification]]== |
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| | ==[[Chronic neutrophilic leukemia pathophysiology|Pathophysiology]]== |
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| The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
| | ==[[Chronic neutrophilic leukemia causes|Causes]]== |
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| | ==[[Chronic neutrophilic leukemia differential diagnosis|Differentiating chronic neutrophilic leukemia from other Diseases]]== |
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| The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
| | ==[[Chronic neutrophilic leukemia epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==Differentiating ((Page name)) from Other Diseases== | | ==[[Chronic neutrophilic leukemia risk factors|Risk Factors]]== |
| [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]. | |
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| | ==[[Chronic neutrophilic leukemia screening|Screening]]== |
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| [Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
| | ==[[Chronic neutrophilic leukemia natural history, compilications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Epidemiology and Demographics== | |
| The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
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| In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
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| In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
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| Patients of all age groups may develop [disease name].
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| The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
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| [Disease name] commonly affects individuals younger than/older than [number of years] years of age.
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| [Chronic disease name] is usually first diagnosed among [age group]. | |
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| [Acute disease name] commonly affects [age group].
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| There is no racial predilection to [disease name].
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| [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
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| [Disease name] affects men and women equally.
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| [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
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| The majority of [disease name] cases are reported in [geographical region].
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| [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
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| ==Risk Factors==
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| There are no established risk factors for [disease name].
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| 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].
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| Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
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| Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
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| ==Screening==
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| There is insufficient evidence to recommend routine screening for [disease/malignancy].
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| According to the [guideline name], screening for [disease name] is not recommended.
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| 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].
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| ==Natural History, Complications, and Prognosis==
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| If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
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| Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
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| Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
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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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| ! style="background: #DCDCDC; text-align: center;" |1. Peripheral blood White blood cells(WBC) ≥25 × 109/L:
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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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| ! 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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| ! 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 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:
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| *Hypercellularity with myeloid hyperplasia
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| in CNL due to expanded neutrophilic granulopoiesis with an increased myeloid to erythroid ratio which may be in excess of 20:128. However, the left shift is distinctly more subtle and mature neutrophils more prominent than in CML with the majority of granulocyte precursors being at the metamyelocyte to segmented stages of maturation. Fewer than 5% myeloblasts are present and there is an absence of Auer rods as well as no dysplastic features16. Reticulin staining, though characteristically normal, may show minimal fibrosis but should not exceed a grade of 1+12. Erythroid maturation is normoblastic and megakaryocytes, while morphologically normal, may be normal or slightly increased in number16.
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| *Increasing of myelocytes, metamyelocytes, and bands
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| *Absence of basophilia and eosinophilia
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| *Megakaryocitic hypeplasia
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| BM aspirates and biopsies show a myeloid hyperplasia (90% cellularity) where myeloblasts represent less than 5% of the cells. Erythro and megakaryopoiesis are typically normal, and dyspoiesis are not present in any cell lineage. Reticulin fibrosis is not significantly increased.CNL co-occurs with monoclonal gammopathy (MG) of undetermined significance in approximately 33% of the cases.2 This phenomenon has been reported in the literature with this subset of 12 patients presenting a MG associated with lambda light chain excess.6,7 However, it remains unclear whether the neutrophilic leukocytosis is a leukemoid response to the underlying MG, or if the presence of the two diseases represents a real entity.8 In cases where the BM shows a plasma cell dyscrasia, it is important to prove the neutrophilic clonality by cytogenetic and/or molecular tests.6
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| The bone marrow is hypercellular with marked myeloid hyperplasia.Myeloid to erythroid ratio is frequently more than 20:1.There is an increase in myelocytes, metamyelocytes and bands, but blasts or promyelocytes are not increased typically (figure 1C, D).Unlike CML, eosinophilia or basophilia are absent. Erythroid precursors are relatively reduced and show normal maturation. In general, megakaryocytes are normal in number and morphology.Some cases may show mild megakaryocytic hyperplasia.7 Significant dyspoiesis is not seen; if present, should prompt one to rule out atypical CML (aCML). Reticulin fibrosis is not observed. Rare cases of CNL have been reported to harbor clonal plasma cells in bone marrow. Bone marrow should be carefully examined for plasma cells, and appropriate immunohistochemical stains should be performed while diagnosing CNL.18 19 In cases where clonal plasma cells are detected, the diagnosis of CNL should be supported by molecular or cytogenetic studies to prove clonality.7
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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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| 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:Up-To-Date]] | | [[Category:Up-To-Date]] |
| | [[Category:Medicine]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Medicine]]
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| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Immunology]] | | [[Category:Immunology]] |