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. Most importantly, other causes of neutropenia should be excluded first.
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