Many patients with FS remain asymptomatic, and the neutropenia may remain undetected unless blood tests are performed to monitor toxicity of anti-rheumatic drugs. Others may present with infections as a result of severe neutropenia, commonly pulmonary or skin infections.

FS is a clinical diagnosis and there is no specific diagnostic test. It is suspected in a patient with long-standing rheumatoid arthritis (RA), associated with neutropenia and frequently (>90% patients) with splenomegaly. Spleen size does not always correlate with the clinical course of the disease or the degree of neutropenia, and so is not considered an absolute diagnostic requirement.[2][4] Most importantly, other causes of neutropenia should be excluded first.

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