Adult-onset Cyclic Neutropenia Diagnosed in a Patient with Acute Arthritis

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.