Case history
Case history
A 65-year-old woman with a 15-year history of seropositive rheumatoid arthritis (RA) with extra-articular manifestations, including leg ulcers and rheumatoid nodules, presents with a several-month history of recurrent sinopulmonary infections. Several blood tests had previously revealed borderline neutropenia. On examination she has mild synovitis of the hands and wrists, and low-grade pyrexia. Blood tests reveal a low neutrophil count, raised erythrocyte sedimentation rate, and strongly positive rheumatoid factor. Blood smear and bone marrow examinations do not reveal features of myelodysplasia, lymphoproliferation, or presence of large granular lymphocytes. Further investigations exclude other causes of neutropenia, such as drug toxicity and opportunistic viral infections. Abdominal ultrasonography reveals splenomegaly and mild hepatomegaly.
Other presentations
The classical triad of FS is RA, neutropenia, and splenomegaly. However, although RA is typically aggressive in patients with FS, synovitis may be mild or inactive at the time of diagnosis.[3] Splenomegaly is frequently found (>90% of patients), but spleen size does not always correlate with clinical course or the degree of neutropenia; splenomegaly is sometimes undetectable on clinical examination or radiological evaluation, despite severe neutropenia.[2][4] Furthermore, many patients with FS do not have recurrent infections and so the neutropenia may remain undetected unless blood tests are performed to monitor for toxicity of anti-rheumatic drugs. In some patients presenting with intercurrent infection, the neutropenia can be masked because active infection can normalise or elevate the white blood cell count.
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