J Rheum Dis.  2013 Feb;20(1):48-51. 10.4078/jrd.2013.20.1.48.

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

• Lee MJ
• Yoo JJ
• Kang EH
• Lee SG
• Shin K
• Lee EY
• Lee EB
• Song YW
• Lee YJ

• KMID: 2223049
• DOI: http://doi.org/10.4078/jrd.2013.20.1.48

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.

Keyword

Cyclic neutropenia; Arthritis

MeSH Terms

Arthralgia
Arthritis
Bacterial Infections
Blood Cell Count
Fever
Granulocyte Colony-Stimulating Factor
Humans
Korea
Leukocyte Elastase
Neutropenia
Periarthritis
Ulcer
Granulocyte Colony-Stimulating Factor
Leukocyte Elastase
Neutropenia

Figure

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  Figure 1. Photomicrograph of bone marrow biopsy. The number of myeloid series decreased without the reduction of megakaryocytes and erythroid precursors in the neutropenic period (A, H&E stain, ×200). However, there was no specific finding after the recovery of neutropenia, except hypocellularity, for his age (B, H&E stain, ×200).

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  Figure 2. Right ankle MRI (sagittal view with contrast enhancement) demonstrated prominent te-nosynovial enhancement along the peroneus longus (filled arrow) and synovial enhancement around the tibiotalar (empty arrow) and subtalar (arrowhead) joint in a T1 fat saturated image (A). Minimal effusion was observed around the tibiotalar (empty arrow) and subtalar (arrowhead) joint in a T2-weighted image (B).

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  Figure 3. Time course of absolute neutrophil counts (ANC) in the present case.

Reference

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