Adult-onset Cyclic Neutropenia Diagnosed in a Patient with Acute Arthritis.
- Author:
Myung Jin LEE
1
;
Jong Jin YOO
;
Eun Ha KANG
;
Sang Guk LEE
;
Kichul SHIN
;
Eun Young LEE
;
Eun Bong LEE
;
Yeong Wook SONG
;
Yun Jong LEE
Author Information
- Publication Type:Case Report
- Keywords: Cyclic neutropenia; Arthritis
- MeSH: Arthralgia; Arthritis; Bacterial Infections; Blood Cell Count; Fever; Granulocyte Colony-Stimulating Factor; Humans; Korea; Leukocyte Elastase; Neutropenia; Periarthritis; Ulcer
- From:Journal of Rheumatic Diseases 2013;20(1):48-51
- CountryRepublic of Korea
- Language:Korean
- Abstract: Cyclic neutropenia (CN) is a rare disorder characterized by repetitive episodes of neutropenia and is generally associated with fever, oral mucosal ulcers, and bacterial infections in the neutropenic episodes. It usually manifests initially in infancy or childhood as an autosomal dominant or sporadic condition; however, adult-onset CN may have an autoimmune etiology. Here, we report the first case of a 22-year old man with CN in Korea. He developed acute arthralgia and fever 4 weeks after an episode of lower gastrointestinal symptoms. Serial blood cell counts showed recurrent neutropenia at 3 week intervals. Further, laboratory examination for neutropenia, including neutrophil elastase gene sequencing, did not reveal any abnormality. His arthritis and periarthritis fluctuated during his course. Under the diagnosis of CN, he received regular G-CSF therapy with partial improvement.
