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Encyclopedia of Medical Immunology pp 1–3Cite as

Cyclic Neutropenia

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Synonyms

Cyclic hematopoiesis; Periodic neutropenia

Definition

Cyclic neutropenia (OMIM #162800) is an autosomal dominant or de novo autosomal disease caused by mutations on the ELANE (formerly known as ELA2) gene that encodes for neutrophil elastase. ELANE gene localization is 19p13.3.

Introduction

Cyclic neutropenia was first mentioned by Reimann and DeBerardinis in the 1940s as they observed a cyclic phenomenon of increase and decrease in neutrophil counts within 18–21 days (Donadieu et al. 2017). In 1999, Horwitz and colleagues identified mutations in the neutrophil elastase gene (ELANE), which were also found in patients with severe congenital neutropenia 1 (SCN1, OMIM #202700). As the same mutation is observed in patients with cyclic neutropenia and permanent SCN and the cyclic aspect is rarely seen in clinical practice, some authors believe that there is a continuum between SCN and intermittent/cyclic neutropenia (Donadieu et al. 2017). Additionally, intermittent neutropenia...

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References

  • Cipe FE, Celiksoy MH, Erturk B, Aydogmus Ç. Cyclic manner of neutropenia in a patient with HAX-1 mutation. Pediatr Hematol Oncol. 2018;35:181–5.

    Article  CAS  Google Scholar 

  • Costa-Carvalho BT, Grumach AS, Franco JL, Espinosa-Rosales FJ, et al. Attending to warning signs of primary immunodeficiency diseases across the range of clinical practice. J Clin Immunol. 2014;34:10–22.

    Article  Google Scholar 

  • Dale DC. How I diagnose and treat neutropenia. Curr Opin Hematol. 2016;23:1–4.

    Article  Google Scholar 

  • Donadieu J, Beaupain B, Fenneteau O, Bellanné-Chantelot C. Congenital neutropenia in the era of genomics: classification, diagnosis, and natural history. Br J Haematol. 2017;179:557–74.

    Article  Google Scholar 

  • Kuruvilla M, de la Morena MT. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract. 2013;1:573–82.

    Article  Google Scholar 

  • Skokowa J, Dale DC, Touw IP, et al. Severe congenital neutropenias. Nat Rev Dis Primers. 2017;3:17032.

    Article  Google Scholar 

  • Sobh A, Bonilla FA. Vaccination in primary immunodeficiency disorders. J Allergy Clin Immunol Pract. 2016;4:1066–75.

    Article  Google Scholar 

  • Spoor J, Farajifard H, Rezaei N. Congenital neutropenia and primary immunodeficiency diseases. Crit Rev Oncol Hematol. 2019;133:149–62.

    Article  Google Scholar 

  • Xia J, Link DC. Severe congenital neutropenia and the unfolded protein response. Curr Opin Hematol. 2008;15:1–7.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Clinical Immunology and Allergy, Department of Clinical Medicine, State University of Campinas (UNICAMP), Campinas, Brazil

    Eli Mansour & Bruna Bombassaro

Authors
  1. Eli Mansour
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  2. Bruna Bombassaro
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Corresponding author

Correspondence to Eli Mansour .

Editor information

Editors and Affiliations

  1. Molecular Biology, Monash University Dept. Biochemistry &, Clayton, VIC, Australia

    Ian MacKay

  2. Autoimmune Disease Research Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Noel R. Rose

Section Editor information

  1. Department of Immunology, Institute of Biomedical Sciences – University of São Paulo, São Paulo, SP, Brazil

    Antonio Condino-Neto

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Mansour, E., Bombassaro, B. (2020). Cyclic Neutropenia. In: MacKay, I., Rose, N. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9209-2_137-1

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  • DOI: https://doi.org/10.1007/978-1-4614-9209-2_137-1

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9209-2

  • Online ISBN: 978-1-4614-9209-2

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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