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__NOTOC__
{{SI}}
{{SI}}
{{CMG}}<br>
{{CMG}} {{AE}} {{Sahar}} <br>
{{SK}} [[Cyclic hematopoiesis]]  
{{SK}} [[Cyclic hematopoiesis]]  
==Overview==
==Overview==
Cyclic neutropenia is a condition in which the [[neutrophil count]] periodically and regularly rises and falls. It is rarely observed in humans, but has been observed in the Grey Collie dog.
Cyclic neutropenia is a [[condition]] in which the [[neutrophil]] count periodically and regularly rises and falls. It is rarely observed in humans, but has been observed in the Grey Collie dog.
==Historical Perspective==
==Historical Perspective==
* Cyclic neutropenia first described in the year 1910 in an infant with recurrent fever.<ref name="DaleBolyard2002">{{cite journal|last1=Dale|first1=David C.|last2=Bolyard|first2=Audrey Anna|last3=Aprikyan|first3=Andrew|title=Cyclic neutropenia|journal=Seminars in Hematology|volume=39|issue=2|year=2002|pages=89–94|issn=00371963|doi=10.1053/shem.2002.31917}}</ref>
* Cyclic neutropenia first described in the year 1910 in an infant with recurrent [[fever]].<ref name="DaleBolyard2002">{{cite journal|last1=Dale|first1=David C.|last2=Bolyard|first2=Audrey Anna|last3=Aprikyan|first3=Andrew|title=Cyclic neutropenia|journal=Seminars in Hematology|volume=39|issue=2|year=2002|pages=89–94|issn=00371963|doi=10.1053/shem.2002.31917}}</ref>
==Classification==
==Classification==
* There is no established system for the [[classification]] of cyclic neutropenia.
* There is no established system for the [[classification]] of cyclic neutropenia.
==Pathophysiology==
==Pathophysiology==
* Cyclic neutropenia is caused by a mutation in the ELA2 gene.<ref name="DaleBolyard2002">{{cite journal|last1=Dale|first1=David C.|last2=Bolyard|first2=Audrey Anna|last3=Aprikyan|first3=Andrew|title=Cyclic neutropenia|journal=Seminars in Hematology|volume=39|issue=2|year=2002|pages=89–94|issn=00371963|doi=10.1053/shem.2002.31917}}</ref><ref name="HorwitzCorey2013">{{cite journal|last1=Horwitz|first1=Marshall S.|last2=Corey|first2=Seth J.|last3=Grimes|first3=H. Leighton|last4=Tidwell|first4=Timothy|title=ELANE Mutations in Cyclic and Severe Congenital Neutropenia|journal=Hematology/Oncology Clinics of North America|volume=27|issue=1|year=2013|pages=19–41|issn=08898588|doi=10.1016/j.hoc.2012.10.004}}</ref>
* Normal [[neutrophil|neutrophilic]] counts is typically between 1500 to 8500 cells/μl after the age of one year.<ref name="ManroeWeinberg1979">{{cite journal|last1=Manroe|first1=Barbara L.|last2=Weinberg|first2=Arthur G.|last3=Rosenfeld|first3=Charles R.|last4=Browne|first4=Richard|title=The neonatal blood count in health and disease.I. Reference values for neutrophilic cells|journal=The Journal of Pediatrics|volume=95|issue=1|year=1979|pages=89–98|issn=00223476|doi=10.1016/S0022-3476(79)80096-7}}</ref>
* The disease occurs in autosomal dominant mode of inheritance.
* Cyclic neutropenia is caused by [[heterozygous]] [[mutation]] in the ELA2 (ELANE) [[gene]].<ref name="DaleBolyard2002">{{cite journal|last1=Dale|first1=David C.|last2=Bolyard|first2=Audrey Anna|last3=Aprikyan|first3=Andrew|title=Cyclic neutropenia|journal=Seminars in Hematology|volume=39|issue=2|year=2002|pages=89–94|issn=00371963|doi=10.1053/shem.2002.31917}}</ref><ref name="HorwitzCorey2013">{{cite journal|last1=Horwitz|first1=Marshall S.|last2=Corey|first2=Seth J.|last3=Grimes|first3=H. Leighton|last4=Tidwell|first4=Timothy|title=ELANE Mutations in Cyclic and Severe Congenital Neutropenia|journal=Hematology/Oncology Clinics of North America|volume=27|issue=1|year=2013|pages=19–41|issn=08898588|doi=10.1016/j.hoc.2012.10.004}}</ref>
* Theoretically, this [[disorder]] can be cured by bone marrow transplantation. This demonstrates its pathogenesis as the stem cell abnormality.<ref name="pmid7036779">{{cite journal |vauthors=Lange RD, Jones JB |title=Cyclic neutropenia. Review of clinical manifestations and management |journal=Am J Pediatr Hematol Oncol |volume=3 |issue=4 |pages=363–7 |date=1981 |pmid=7036779 |doi= |url=}}</ref>  
* The [[disease]] occurs in [[autosomal dominant]] mode of inheritance.
* This stem cell abnormality leads to the myelocyte maturation arrest during neutropenia episodes.
* The culprit [[gene]] is responsible for encoding the [[neutrophil]] granule serine protease, neutrophil elastase.
* [[Mutation]] results in abnormal [[gene]] product that damages cells while they mature, leading to to failure of [[cell]] production.
* Theoretically, this [[disorder]] can be cured by [[bone marrow transplantation]]. This demonstrates its [[pathogenesis]]] as the [[stem cell]] abnormality.<ref name="pmid7036779">{{cite journal |vauthors=Lange RD, Jones JB |title=Cyclic neutropenia. Review of clinical manifestations and management |journal=Am J Pediatr Hematol Oncol |volume=3 |issue=4 |pages=363–7 |date=1981 |pmid=7036779 |doi= |url=}}</ref>
* This [[stem cell]] abnormality leads to the myelocyte maturation arrest during [[neutropenia]] episodes.
==Causes==
==Causes==
* Cyclic neutropenia is caused by a mutation in the ELA2 gene.<ref name="HorwitzCorey2013">{{cite journal|last1=Horwitz|first1=Marshall S.|last2=Corey|first2=Seth J.|last3=Grimes|first3=H. Leighton|last4=Tidwell|first4=Timothy|title=ELANE Mutations in Cyclic and Severe Congenital Neutropenia|journal=Hematology/Oncology Clinics of North America|volume=27|issue=1|year=2013|pages=19–41|issn=08898588|doi=10.1016/j.hoc.2012.10.004}}</ref>
* Cyclic neutropenia is caused by [[heterozygous]] mutation in the ELA2 (ELANE) [[gene]].<ref name="HorwitzCorey2013">{{cite journal|last1=Horwitz|first1=Marshall S.|last2=Corey|first2=Seth J.|last3=Grimes|first3=H. Leighton|last4=Tidwell|first4=Timothy|title=ELANE Mutations in Cyclic and Severe Congenital Neutropenia|journal=Hematology/Oncology Clinics of North America|volume=27|issue=1|year=2013|pages=19–41|issn=08898588|doi=10.1016/j.hoc.2012.10.004}}</ref>
==Differentiating Cyclic neutropenia from Other Diseases==
==Differentiating Cyclic neutropenia from Other Diseases==
* Cyclic neutropenia should be differentiated from other disorders manifesting with recurrent [[fever]], [[aphthous stomatitis]], and [[pharyngitis]]. These include [[PFAPA syndrome]], and some of the monogenic [[autoinflammatory disorders]] such as [[FMF]], [[CAPS]], and [[TRAPS]].<ref name="AliSartori-Valinotti2016">{{cite journal|last1=Ali|first1=Nora S.|last2=Sartori-Valinotti|first2=Julio C.|last3=Bruce|first3=Alison J.|title=Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome|journal=Clinics in Dermatology|volume=34|issue=4|year=2016|pages=482–486|issn=0738081X|doi=10.1016/j.clindermatol.2016.02.021}}</ref>
* Cyclic neutropenia should be differentiated from other [[disorders]] manifesting with recurrent [[fever]], [[aphthous stomatitis]], and [[pharyngitis]]. These include [[PFAPA syndrome]], monogenic [[autoinflammatory disorders]] such as [[FMF]], [[CAPS]], and [[TRAPS]], as well as primary [[immunodeficiencies]].<ref name="AliSartori-Valinotti2016">{{cite journal|last1=Ali|first1=Nora S.|last2=Sartori-Valinotti|first2=Julio C.|last3=Bruce|first3=Alison J.|title=Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome|journal=Clinics in Dermatology|volume=34|issue=4|year=2016|pages=482–486|issn=0738081X|doi=10.1016/j.clindermatol.2016.02.021}}</ref>
* For more information on cyclic neutropenia [[differential diagnosis]] please [[Familial mediterranean fever differential diagnosis|click here]].
*[[Shwachman-Diamond syndrome]] may also present with [[neutropenia]]. However, its [[diagnosis]] requires some [[dysmorphic]] features which are not present in patients with cyclic neutropenia.<ref name="pmid10393609">{{cite journal |vauthors=Ginzberg H, Shin J, Ellis L, Morrison J, Ip W, Dror Y, Freedman M, Heitlinger LA, Belt MA, Corey M, Rommens JM, Durie PR |title=Shwachman syndrome: phenotypic manifestations of sibling sets and isolated cases in a large patient cohort are similar |journal=J. Pediatr. |volume=135 |issue=1 |pages=81–8 |date=July 1999 |pmid=10393609 |doi=10.1016/s0022-3476(99)70332-x |url=}}</ref>
* For more information on cyclic neutropenia [[differential diagnosis]] please [[Neutropenia differential diagnosis|click here]].
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The incidence of cyclic neutropenia is 0.010-0.02 per 100,000 individuals worldwide.<ref name="pmid14962902">{{cite journal |vauthors=Bellanné-Chantelot C, Clauin S, Leblanc T, Cassinat B, Rodrigues-Lima F, Beaufils S, Vaury C, Barkaoui M, Fenneteau O, Maier-Redelsperger M, Chomienne C, Donadieu J |title=Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register |journal=Blood |volume=103 |issue=11 |pages=4119–25 |date=June 2004 |pmid=14962902 |doi=10.1182/blood-2003-10-3518 |url=}}</ref>
* The incidence of cyclic neutropenia is 0.010-0.02 per 100,000 individuals worldwide.<ref name="pmid14962902">{{cite journal |vauthors=Bellanné-Chantelot C, Clauin S, Leblanc T, Cassinat B, Rodrigues-Lima F, Beaufils S, Vaury C, Barkaoui M, Fenneteau O, Maier-Redelsperger M, Chomienne C, Donadieu J |title=Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register |journal=Blood |volume=103 |issue=11 |pages=4119–25 |date=June 2004 |pmid=14962902 |doi=10.1182/blood-2003-10-3518 |url=}}</ref>
* There is no [[racial]] predilection to cyclic neutropenia.
* Cyclic neutropenia affects [[men]] and [[women]] equally.
==Risk Factors==
==Risk Factors==
* There are no established [[risk factors]] for cyclic neutropenia.
* There are no established [[risk factors]] for cyclic neutropenia.
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==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
* Disease manifests in early years of life with episodes of [[fever]] occurring every 21 days (range from 14 to 35 days).<ref name="pmid11957190">{{cite journal |vauthors=Dale DC, Bolyard AA, Aprikyan A |title=Cyclic neutropenia |journal=Semin. Hematol. |volume=39 |issue=2 |pages=89–94 |date=April 2002 |pmid=11957190 |doi= |url=}}</ref>
* Disease manifests in early years of life with episodes of [[fever]] occurring every 21 days (range from 14 to 35 days).<ref name="pmid11957190">{{cite journal |vauthors=Dale DC, Bolyard AA, Aprikyan A |title=Cyclic neutropenia |journal=Semin. Hematol. |volume=39 |issue=2 |pages=89–94 |date=April 2002 |pmid=11957190 |doi= |url=}}</ref>
* The hallmark of this disorder is the predictability of the fever episodes.   
* The hallmark of this [[disorder]] is the predictability of the fever episodes.   
* Patients may also present with gingivitis, stomatitis, and malaise.
* Patients may be asymptomatic or develop life-threatening infections depending on the severity of neutropenia.
* During episodes, patients are neutropenic.  
* During episodes, [[patients]] are neutropenic and it increases their risk for [[dental]] and [[gingival]] [[complications]].<ref name="PalmerStephens1996">{{cite journal|last1=Palmer|first1=Susan E.|last2=Stephens|first2=Karen|last3=Dale|first3=David C.|title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis|journal=American Journal of Medical Genetics|volume=66|issue=4|year=1996|pages=413–422|issn=01487299|doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L}}</ref>
* Although [[patients]] with cyclic neutropenia are not as immunodeficient as the post-chemotherapy [[patients]], they still should not be assumed normal.
* Serious [[complications]] of this disorder include [[pneumonia]], [[mastoiditis]], and [[bacterial]] [[cutaneous]] and [[subcutaneous infections]].<ref name="GlavanRoganović2015">{{cite journal|last1=Glavan|first1=Nedeljka|last2=Roganović|first2=Jelena|last3=Glavan-Gacanin|first3=Lana|last4=Jonjic|first4=Nives|title=Appendectomy in a child with cyclic neutropenia in profound neutropenic episode|journal=Therapeutics and Clinical Risk Management|year=2015|pages=1217|issn=1178-203X|doi=10.2147/TCRM.S89488}}</ref>
* Death induced by [[infections]] are a possible [[complication]] of this [[disorder]] and has been reported in 10% of the [[patients]].<ref name="DaleCottle2003">{{cite journal|last1=Dale|first1=David C.|last2=Cottle|first2=Tammy E.|last3=Fier|first3=Carol J.|last4=Bolyard|first4=Audrey Anna|last5=Bonilla|first5=Mary Ann|last6=Boxer|first6=Laurence A.|last7=Cham|first7=Bonnie|last8=Freedman|first8=Melvin H.|last9=Kannourakis|first9=George|last10=Kinsey|first10=Sally E.|last11=Davis|first11=Robert|last12=Scarlata|first12=Debra|last13=Schwinzer|first13=Beate|last14=Zeidler|first14=Cornelia|last15=Welte|first15=Karl|title=Severe chronic neutropenia: Treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry|journal=American Journal of Hematology|volume=72|issue=2|year=2003|pages=82–93|issn=0361-8609|doi=10.1002/ajh.10255}}</ref>
==Diagnosis==
==Diagnosis==
* [[Diagnosis]] of cyclic neutropenia is based on the clinical picture and the exclusion of other possible [[causes]] of [[neutropenia]].<ref name="pmid11957190">{{cite journal |vauthors=Dale DC, Bolyard AA, Aprikyan A |title=Cyclic neutropenia |journal=Semin. Hematol. |volume=39 |issue=2 |pages=89–94 |date=April 2002 |pmid=11957190 |doi= |url=}}</ref>
*[[ANC|Absolute necrophiliac count]] ([[ANC]]) should be <200/microL, usually for at least two to three days, in each of two to three regularly spaced cycles.
* [[Genetic analysis]] may be done for the confirmation of the [[diagnosis]]. However, it usually turns positive for [[gene mutation]] in 90% of the [[patients]].<ref name="pmid17053055">{{cite journal |vauthors=Horwitz MS, Duan Z, Korkmaz B, Lee HH, Mealiffe ME, Salipante SJ |title=Neutrophil elastase in cyclic and severe congenital neutropenia |journal=Blood |volume=109 |issue=5 |pages=1817–24 |date=March 2007 |pmid=17053055 |pmc=1801070 |doi=10.1182/blood-2006-08-019166 |url=}}</ref>
* [[Bone marrow aspiration]] is not considered helpful for the [[diagnosis]].
===Diagnostic Criteria===
===Diagnostic Criteria===
* There is no [[diagnostic]] criteria for the [[diagnosis]] of cyclic neutropenia.
===History and Symptoms===
===History and Symptoms===
* Symptoms of cyclic neutropenia include fever, malaise, oral ulcers, gingival inflammation, edema, and sore throat.<ref name="pmid8989458">{{cite journal |vauthors=Palmer SE, Stephens K, Dale DC |title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis |journal=Am. J. Med. Genet. |volume=66 |issue=4 |pages=413–22 |date=December 1996 |pmid=8989458 |doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L |url=}}</ref><ref name="pmid7036779">{{cite journal |vauthors=Lange RD, Jones JB |title=Cyclic neutropenia. Review of clinical manifestations and management |journal=Am J Pediatr Hematol Oncol |volume=3 |issue=4 |pages=363–7 |date=1981 |pmid=7036779 |doi= |url=}}</ref>
* [[Symptoms]] of cyclic neutropenia include [[fever]], [[malaise]], [[oral ulcers]], [[gingival]] [[inflammation]], edema, and [[sore throat]].<ref name="pmid8989458">{{cite journal |vauthors=Palmer SE, Stephens K, Dale DC |title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis |journal=Am. J. Med. Genet. |volume=66 |issue=4 |pages=413–22 |date=December 1996 |pmid=8989458 |doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L |url=}}</ref><ref name="pmid7036779">{{cite journal |vauthors=Lange RD, Jones JB |title=Cyclic neutropenia. Review of clinical manifestations and management |journal=Am J Pediatr Hematol Oncol |volume=3 |issue=4 |pages=363–7 |date=1981 |pmid=7036779 |doi= |url=}}</ref>
===Physical Examination===
===Physical Examination===
* Physical examination of patients with cyclic neutropenia include:<ref name="pmid8989458">{{cite journal |vauthors=Palmer SE, Stephens K, Dale DC |title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis |journal=Am. J. Med. Genet. |volume=66 |issue=4 |pages=413–22 |date=December 1996 |pmid=8989458 |doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L |url=}}</ref>
* [[Physical examination]] of [[patients]] with cyclic neutropenia include [[fever]], [[pharyngitis]], and [[gingival]] [[inflammation]] and [[edema]].<ref name="pmid8989458">{{cite journal |vauthors=Palmer SE, Stephens K, Dale DC |title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis |journal=Am. J. Med. Genet. |volume=66 |issue=4 |pages=413–22 |date=December 1996 |pmid=8989458 |doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L |url=}}</ref>
** Fever
 
** Pharyngitis
** Gingival inflammation and edema
===Laboratory Findings===
===Laboratory Findings===
* Laboratory findings associated with the diagnosis of cyclic neutropenia include:
* Laboratory findings associated with the [[diagnosis]] of cyclic neutropenia include:<ref name="GlavanRoganović2015">{{cite journal|last1=Glavan|first1=Nedeljka|last2=Roganović|first2=Jelena|last3=Glavan-Gacanin|first3=Lana|last4=Jonjic|first4=Nives|title=Appendectomy in a child with cyclic neutropenia in profound neutropenic episode|journal=Therapeutics and Clinical Risk Management|year=2015|pages=1217|issn=1178-203X|doi=10.2147/TCRM.S89488}}</ref>
** Low absolute neutrophilic count
** Low [[absolute neutrophilic count]]
**[[Neutropenia]] may be mild or severe with a nadir of 100/μL for 3–6 days in each cycle. However, the duration of [[neutropenia]] varies between the [[patients]].
===Electrocardiogram===
===Electrocardiogram===
* There are no [[ECG]] findings associated with cyclic neutropenia.
* There are no [[ECG]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===X-ray===
===X-ray===
* There are no [[x-ray]] findings associated with cyclic neutropenia.
* There are no [[x-ray]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
* There are no [[echocardiography]]/[[ultrasound]] findings associated with cyclic neutropenia.
* There are no [[echocardiography]]/[[ultrasound]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===CT Scan===
===CT Scan===
* There are no [[CT scan]] findings associated with cyclic neutropenia.
* There are no [[CT scan]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===MRI===
===MRI===
* There are no [[MRI]] findings associated with cyclic neutropenia.
* There are no [[MRI]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===Other Imaging Findings===
===Other Imaging Findings===
* There are no other [[imaging]] findings associated with cyclic neutropenia.
* There are no other [[imaging]] findings [[Association (statistics)|associated]] with cyclic neutropenia.
===Other Diagnostic Studies===
===Other Diagnostic Studies===
* There are no other [[diagnostic]] studies [[Association (statistics)|associated]] with cyclic neutropenia.
* There are no other [[diagnostic]] studies [[Association (statistics)|associated]] with cyclic neutropenia.
==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
* The mainstay of treatment for cyclic neutropenia is medical therapy.<ref name="pmid2469956">{{cite journal |vauthors=Hammond WP, Price TH, Souza LM, Dale DC |title=Treatment of cyclic neutropenia with granulocyte colony-stimulating factor |journal=N. Engl. J. Med. |volume=320 |issue=20 |pages=1306–11 |date=May 1989 |pmid=2469956 |doi=10.1056/NEJM198905183202003 |url=}}</ref><ref name="pmid7529539">{{cite journal |vauthors=Bonilla MA, Dale D, Zeidler C, Last L, Reiter A, Ruggeiro M, Davis M, Koci B, Hammond W, Gillio A |title=Long-term safety of treatment with recombinant human granulocyte colony-stimulating factor (r-metHuG-CSF) in patients with severe congenital neutropenias |journal=Br. J. Haematol. |volume=88 |issue=4 |pages=723–30 |date=December 1994 |pmid=7529539 |doi=10.1111/j.1365-2141.1994.tb05110.x |url=}}</ref>
* The mainstay of treatment for cyclic neutropenia is medical therapy.<ref name="pmid2469956">{{cite journal |vauthors=Hammond WP, Price TH, Souza LM, Dale DC |title=Treatment of cyclic neutropenia with granulocyte colony-stimulating factor |journal=N. Engl. J. Med. |volume=320 |issue=20 |pages=1306–11 |date=May 1989 |pmid=2469956 |doi=10.1056/NEJM198905183202003 |url=}}</ref><ref name="pmid7529539">{{cite journal |vauthors=Bonilla MA, Dale D, Zeidler C, Last L, Reiter A, Ruggeiro M, Davis M, Koci B, Hammond W, Gillio A |title=Long-term safety of treatment with recombinant human granulocyte colony-stimulating factor (r-metHuG-CSF) in patients with severe congenital neutropenias |journal=Br. J. Haematol. |volume=88 |issue=4 |pages=723–30 |date=December 1994 |pmid=7529539 |doi=10.1111/j.1365-2141.1994.tb05110.x |url=}}</ref>
* Granulocyte colony-stimulating factor (G-CSF) may be used to treat cyclic neutropenia.  
* It is recommended that all [[patients]] receive [[Granulocyte colony-stimulating factor]] ([[G-CSF]]). However, the mainstay of treatment is supportive.<ref name="pmid8989458">{{cite journal |vauthors=Palmer SE, Stephens K, Dale DC |title=Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis |journal=Am. J. Med. Genet. |volume=66 |issue=4 |pages=413–22 |date=December 1996 |pmid=8989458 |doi=10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L |url=}}</ref><ref name="pmid8589368">{{cite journal |vauthors=Heussner P, Haase D, Kanz L, Fonatsch C, Welte K, Freund M |title=G-CSF in the long-term treatment of cyclic neutropenia and chronic idiopathic neutropenia in adult patients |journal=Int. J. Hematol. |volume=62 |issue=4 |pages=225–34 |date=December 1995 |pmid=8589368 |doi= |url=}}</ref>
*[[G-CSF]] should be administered [[Subcutaneous|subcutaneously]] starting at 2 to 3 mcg/kg every one to two days.
* [[Patients]] with following features should receive [[G-CSF]]:
**[[Patients]] with recurrent [[symptoms]]
** [[Patients]] with evidence of [[gingival]] disease or severe [[infection]]
* Regular [[dental]] care is recommended.  
===Surgery===
===Surgery===
* Surgical intervention is not recommended for the management of cyclic neutropenia.
*[[Surgical]] intervention is not recommended for the [[management]] of cyclic neutropenia.
===Primary Prevention===
===Primary Prevention===
* There are no established measures for the [[primary prevention]] of cyclic neutropenia.
* There are no established measures for the [[primary prevention]] of cyclic neutropenia.
===Secondary Prevention===
===Secondary Prevention===
* There are no established measures for the [[secondary prevention]] of cyclic neutropenia.
* There are no established measures for the [[secondary prevention]] of cyclic neutropenia.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Hematology]]
[[Category:Hematology]]
{{WS}}
{{WH}}

Latest revision as of 03:09, 23 September 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Synonyms and keywords: Cyclic hematopoiesis

Overview

Cyclic neutropenia is a condition in which the neutrophil count periodically and regularly rises and falls. It is rarely observed in humans, but has been observed in the Grey Collie dog.

Historical Perspective

  • Cyclic neutropenia first described in the year 1910 in an infant with recurrent fever.[1]

Classification

  • There is no established system for the classification of cyclic neutropenia.

Pathophysiology

Causes

Differentiating Cyclic neutropenia from Other Diseases

Epidemiology and Demographics

  • The incidence of cyclic neutropenia is 0.010-0.02 per 100,000 individuals worldwide.[7]
  • There is no racial predilection to cyclic neutropenia.
  • Cyclic neutropenia affects men and women equally.

Risk Factors

  • There are no established risk factors for cyclic neutropenia.

Screening

  • There is insufficient evidence to recommend routine screening for cyclic neutropenia.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography or Ultrasound

CT Scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

  1. 1.0 1.1 Dale, David C.; Bolyard, Audrey Anna; Aprikyan, Andrew (2002). "Cyclic neutropenia". Seminars in Hematology. 39 (2): 89–94. doi:10.1053/shem.2002.31917. ISSN 0037-1963.
  2. Manroe, Barbara L.; Weinberg, Arthur G.; Rosenfeld, Charles R.; Browne, Richard (1979). "The neonatal blood count in health and disease.I. Reference values for neutrophilic cells". The Journal of Pediatrics. 95 (1): 89–98. doi:10.1016/S0022-3476(79)80096-7. ISSN 0022-3476.
  3. 3.0 3.1 Horwitz, Marshall S.; Corey, Seth J.; Grimes, H. Leighton; Tidwell, Timothy (2013). "ELANE Mutations in Cyclic and Severe Congenital Neutropenia". Hematology/Oncology Clinics of North America. 27 (1): 19–41. doi:10.1016/j.hoc.2012.10.004. ISSN 0889-8588.
  4. 4.0 4.1 Lange RD, Jones JB (1981). "Cyclic neutropenia. Review of clinical manifestations and management". Am J Pediatr Hematol Oncol. 3 (4): 363–7. PMID 7036779.
  5. Ali, Nora S.; Sartori-Valinotti, Julio C.; Bruce, Alison J. (2016). "Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome". Clinics in Dermatology. 34 (4): 482–486. doi:10.1016/j.clindermatol.2016.02.021. ISSN 0738-081X.
  6. Ginzberg H, Shin J, Ellis L, Morrison J, Ip W, Dror Y, Freedman M, Heitlinger LA, Belt MA, Corey M, Rommens JM, Durie PR (July 1999). "Shwachman syndrome: phenotypic manifestations of sibling sets and isolated cases in a large patient cohort are similar". J. Pediatr. 135 (1): 81–8. doi:10.1016/s0022-3476(99)70332-x. PMID 10393609.
  7. Bellanné-Chantelot C, Clauin S, Leblanc T, Cassinat B, Rodrigues-Lima F, Beaufils S, Vaury C, Barkaoui M, Fenneteau O, Maier-Redelsperger M, Chomienne C, Donadieu J (June 2004). "Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register". Blood. 103 (11): 4119–25. doi:10.1182/blood-2003-10-3518. PMID 14962902.
  8. 8.0 8.1 Dale DC, Bolyard AA, Aprikyan A (April 2002). "Cyclic neutropenia". Semin. Hematol. 39 (2): 89–94. PMID 11957190.
  9. Palmer, Susan E.; Stephens, Karen; Dale, David C. (1996). "Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis". American Journal of Medical Genetics. 66 (4): 413–422. doi:10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L. ISSN 0148-7299.
  10. 10.0 10.1 Glavan, Nedeljka; Roganović, Jelena; Glavan-Gacanin, Lana; Jonjic, Nives (2015). "Appendectomy in a child with cyclic neutropenia in profound neutropenic episode". Therapeutics and Clinical Risk Management: 1217. doi:10.2147/TCRM.S89488. ISSN 1178-203X.
  11. Dale, David C.; Cottle, Tammy E.; Fier, Carol J.; Bolyard, Audrey Anna; Bonilla, Mary Ann; Boxer, Laurence A.; Cham, Bonnie; Freedman, Melvin H.; Kannourakis, George; Kinsey, Sally E.; Davis, Robert; Scarlata, Debra; Schwinzer, Beate; Zeidler, Cornelia; Welte, Karl (2003). "Severe chronic neutropenia: Treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry". American Journal of Hematology. 72 (2): 82–93. doi:10.1002/ajh.10255. ISSN 0361-8609.
  12. Horwitz MS, Duan Z, Korkmaz B, Lee HH, Mealiffe ME, Salipante SJ (March 2007). "Neutrophil elastase in cyclic and severe congenital neutropenia". Blood. 109 (5): 1817–24. doi:10.1182/blood-2006-08-019166. PMC 1801070. PMID 17053055.
  13. 13.0 13.1 13.2 Palmer SE, Stephens K, Dale DC (December 1996). "Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis". Am. J. Med. Genet. 66 (4): 413–22. doi:10.1002/(SICI)1096-8628(19961230)66:4<413::AID-AJMG5>3.0.CO;2-L. PMID 8989458.
  14. Hammond WP, Price TH, Souza LM, Dale DC (May 1989). "Treatment of cyclic neutropenia with granulocyte colony-stimulating factor". N. Engl. J. Med. 320 (20): 1306–11. doi:10.1056/NEJM198905183202003. PMID 2469956.
  15. Bonilla MA, Dale D, Zeidler C, Last L, Reiter A, Ruggeiro M, Davis M, Koci B, Hammond W, Gillio A (December 1994). "Long-term safety of treatment with recombinant human granulocyte colony-stimulating factor (r-metHuG-CSF) in patients with severe congenital neutropenias". Br. J. Haematol. 88 (4): 723–30. doi:10.1111/j.1365-2141.1994.tb05110.x. PMID 7529539.
  16. Heussner P, Haase D, Kanz L, Fonatsch C, Welte K, Freund M (December 1995). "G-CSF in the long-term treatment of cyclic neutropenia and chronic idiopathic neutropenia in adult patients". Int. J. Hematol. 62 (4): 225–34. PMID 8589368.