Non-Articular Felty Syndrome Refractory to Granulocyte Colony-Stimulating Factor Therapy

Felty syndrome (FS), an uncommon manifestation seen in patients with rheumatoid arthritis (RA), usually presents as a triad of erosive arthritis, splenomegaly, and neutropenia. It is extremely rare for RA to present as FS or develop after initially presenting as neutropenia and splenomegaly. In this report, we describe a case of a 55-year-old woman who initially presented with fever and vaginal pain. Her sepsis workup revealed genital herpes in the setting of leukopenia, with an incidental finding of splenomegaly on imaging. The patient was managed with filgrastim and valacyclovir. Two weeks later, she presented again with pleuritic chest pain and worsening leukopenia. This led to an extensive workup by the hematology team to diagnose and confirm the diagnosis of FS. We also engage in a review of the existing literature of such cases and emphasize the importance of serological testing for RA in patients with leukopenia and splenomegaly, even in the absence of joint symptoms or prior diagnosis of RA. The management should be guided towards treating the infection, correcting the neutropenia, and treating the underlying chronic disease.