급성관절염 환자에서 진단된 성인형 주기성 호중구감소증 1예 (Adult-onset Cyclic Neutropenia Diagnosed in a Patient with Acute Arthritis)

서지정보

· 발행기관 : 대한류마티스학회
· 수록지 정보 : 대한류마티스학회지 / 20권 / 1호 / 48 ~ 51페이지
· 저자명 : 이명진, 유종진, 강은하, 이상국, 신기철, 이은영, 이은봉, 송영욱, 이윤종

초록

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.

영어초록

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.

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