Cyclic neutropenia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
{SK} 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

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.[2]

Risk Factors

Screening

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).[3]
  • The hallmark of this disorder is the predictability of the fever episodes.
  • Patients may also present with gingivitis, stomatitis, and malaise.
  • During episodes, patients are neutropenic.

Diagnosis

Diagnostic Criteria

History and Symptoms

  • Symptoms of cyclic neutropenia include fever, malaise, oral ulcers, gingival inflammation, edema, and sore throat.[4]

Physical Examination

  • Physical examination of patients with cyclic neutropenia include:[4]
    • Fever
    • Pharyngitis
    • Gingival inflammation and edema

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  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. 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.
  3. Dale DC, Bolyard AA, Aprikyan A (April 2002). "Cyclic neutropenia". Semin. Hematol. 39 (2): 89–94. PMID 11957190.
  4. 4.0 4.1 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.

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