Cyclic neutropenia

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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


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]


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



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.


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

Natural History, Complications, and Prognosis


Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings



Echocardiography or Ultrasound

CT Scan


Other Imaging Findings

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention


  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.