Cyclic neutropenia: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
No edit summary |
||
| Line 1: | Line 1: | ||
{{SI}} | {{SI}} | ||
{{CMG}} | {{CMG}}<br> | ||
{SK} Cyclic hematopoiesis | |||
==Overview== | ==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. | 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. | ||
| Line 12: | Line 12: | ||
==Causes== | ==Causes== | ||
==Differentiating | ==Differentiating Cyclic neutropenia from Other Diseases== | ||
==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> | |||
==Risk Factors== | ==Risk Factors== | ||
| Line 21: | Line 21: | ||
==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> | ||
* 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== | ==Diagnosis== | ||
===Diagnostic Criteria=== | ===Diagnostic Criteria=== | ||
===History and Symptoms=== | ===History and Symptoms=== | ||
* Symptoms of cyclic neutropenia include fever, malaise, oral ulcers, gingival inflammation, edema, and sore throat.<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=== | ===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> | |||
** Fever | |||
** Pharyngitis | |||
** Gingival inflammation and edema | |||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Revision as of 15:18, 27 August 2019
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
- ↑ 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.