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Felty's Syndrome Presenting as Neutropenic Fever
Felty's Syndrome Presenting as Neutropenic Fever
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This case describes a 56-year-old woman with hypertension and type 2 diabetes who presented to the emergency department with fever and confusion. She was found to have severe neutropenia, anemia, thrombocytopenia, and splenomegaly. Initial evaluation for infection and other common causes of neutropenic fever was negative, and prior bone marrow biopsies had shown no malignancy.<br /><br />Because of the combination of neutropenia and splenomegaly, rheumatologic testing was pursued. Results were strongly suggestive of rheumatoid arthritis, with elevated rheumatoid factor, anti-CCP antibodies, positive ANA, and low complement. CT imaging confirmed marked splenomegaly, and flow cytometry showed an atypical T-cell population, raising concern for Felty’s syndrome versus large granular lymphocytic leukemia. Further oncology evaluation, including repeat bone marrow biopsy and myelodysplastic syndrome testing, did not reveal cancer or another hematologic malignancy.<br /><br />The patient’s fever and confusion improved with antibiotics and supportive care, and her neutrophil count recovered after G-CSF. She was discharged on prednisone and methotrexate but was readmitted one month later with recurrent neutropenic fever. She ultimately underwent splenectomy, after which her white blood cell counts improved over the following months and she had no further episodes of neutropenia.<br /><br />Felty’s syndrome is classically defined by the triad of neutropenia, splenomegaly, and rheumatoid arthritis, usually occurring late in the course of long-standing RA. Neutropenia may be caused by immune mechanisms, including possible anti–G-CSF antibodies, and by splenic sequestration. Because large granular lymphocytic leukemia can present similarly, evaluation should include flow cytometry and bone marrow studies. Treatment typically begins with disease-modifying antirheumatic drugs, with splenectomy reserved for refractory cases. This case highlights the importance of considering Felty’s syndrome in patients with neutropenic fever, especially when splenomegaly and serologic evidence of rheumatoid arthritis are present.
Asset Subtitle
Victor E. Serrano Santiago
Meta Tag
Author List
Victor E. Serrano Santiago, Zack J. Morgan
Category
Clinical Vignettes
Concept
Felty syndrome
Concept
Neutropenia
Concept
Splenomegaly
Concept
Rheumatoid Arthritis
Concept
Neutropenic Fever
Distinguished
Non-Finalist
Presenter Organization
Methodist Dallas Medical Center
Presenting Author
Victor E. Serrano Santiago
Track
Adult
Keywords
Felty syndrome
neutropenic fever
splenomegaly
rheumatoid arthritis
severe neutropenia
anti-CCP antibodies
rheumatoid factor
large granular lymphocytic leukemia
splenectomy
methotrexate
Felty syndrome
Neutropenia
Splenomegaly
Rheumatoid Arthritis
Neutropenic Fever
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