Cyclic neutropenia: Difference between revisions
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* 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. | |||
==Pathophysiology== | ==Pathophysiology== | ||
| Line 17: | Line 17: | ||
* 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> | ||
==Risk Factors== | ==Risk Factors== | ||
* There are no established [[risk factors]] for cyclic neutropenia. | |||
==Screening== | ==Screening== | ||
* There is insufficient evidence to recommend routine [[screening]] for cyclic neutropenia. | |||
==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> | ||
Revision as of 15:34, 27 August 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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.[2]
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
- 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
- Laboratory findings associated with the diagnosis of cyclic neutropenia include:
- Low absolute neutrophilic count
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
- There are no echocardiography/ultrasound findings associated with cyclic neutropenia.
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
- There are no other diagnostic studies associated with cyclic neutropenia.
Treatment
Medical Therapy
Surgery
- Surgical intervention is not recommended for the management of cyclic neutropenia.
Primary Prevention
- There are no established measures for the primary prevention of cyclic neutropenia.
Secondary Prevention
- There are no established measures for the secondary prevention of cyclic neutropenia.
References
- ↑ 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.
- ↑ 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.
- ↑ Dale DC, Bolyard AA, Aprikyan A (April 2002). "Cyclic neutropenia". Semin. Hematol. 39 (2): 89–94. PMID 11957190.
- ↑ 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.