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* 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 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>
* 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>
* The disease occurs in autosomal dominant mode of inheritance.
* The disease occurs in autosomal dominant mode of inheritance.
* 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 te 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>  
* 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.
* 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]], monogenic [[autoinflammatory disorders]] such as [[FMF]], [[CAPS]], and [[TRAPS]], as well as primary immunodeficincies.<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 immunodeficincies.<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>
* 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>
* 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]].
* 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>
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* 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 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>
* 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.
* 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>
* 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]].
* 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==
===Diagnostic Criteria===
===Diagnostic Criteria===
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===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:<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
** [[Fever]]
** Pharyngitis
** [[Pharyngitis]]
** Gingival inflammation and edema
** [[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, th duration of neutropenia varies between the [[patients]].
===Electrocardiogram===
===Electrocardiogram===
* There are no [[ECG]] findings associated with cyclic neutropenia.
* There are no [[ECG]] findings associated with cyclic neutropenia.

Revision as of 16:12, 25 September 2019

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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

  • Normal neutrophilic counts is typically between 1500 to 8500 cells/μl after the age of one year.[2]
  • Cyclic neutropenia is caused by heterozygous mutation in the ELA2 (ELANE) gene.[1][3]
  • The disease occurs in autosomal dominant mode of inheritance.
  • 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 te failure of cell production.
  • Theoretically, this disorder can be cured by bone marrow transplantation. This demonstrates its pathogenesis as the stem cell abnormality.[4]
  • This stem cell abnormality leads to the myelocyte maturation arrest during neutropenia episodes.

Causes

  • Cyclic neutropenia is caused by heterozygous mutation in the ELA2 (ELANE) gene.[3]

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

  • Laboratory findings associated with the diagnosis of cyclic neutropenia include:[10]
    • Low absolute neutrophilic count
    • Neutropenia may be mild or severe with a nadir of 100/μL for 3–6 days in each cycle. However, th duration of neutropenia varies between the patients.

Electrocardiogram

  • There are no ECG findings associated with cyclic neutropenia.

X-ray

  • There are no x-ray findings associated with cyclic neutropenia.

Echocardiography or Ultrasound

CT Scan

  • There are no CT scan findings associated with cyclic neutropenia.

MRI

  • There are no MRI findings associated with cyclic neutropenia.

Other Imaging Findings

  • There are no other imaging findings associated with cyclic neutropenia.

Other Diagnostic Studies

Treatment

Medical Therapy

  • The mainstay of treatment for cyclic neutropenia is medical therapy.[13][14]
  • Granulocyte colony-stimulating factor (G-CSF) may be used to treat cyclic neutropenia.

Surgery

  • Surgical intervention is not recommended for the management of cyclic neutropenia.

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. 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. 12.0 12.1 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.
  13. 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.
  14. 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.

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