What three findings make up the Classic triad of Feltys syndrome?
Felty’s syndrome is RA in combination with splenomegaly and leukopenia. Felty’s syndrome is seen in 1% of RA patients who have RF, subcutaneous nodules, and other extraarticular manifestations. Most (95%) of patients are HLA-DR4- and RF-positive. Articular disease parallels those of RF-positive patients, but Felty’s syndrome patients have more extraarticular manifestations. The leukopenia is generally a neutropenia (<2000/mm 3 ); thrombocytopenia may occur. The major complications of Felty’s syndrome include bacterial infections (20-fold increase compared with other RA patients) and chronic non-healing ulcers. Severe bacterial infections correlate with neutrophil counts of <1000/mm 3 . Patients with Felty’s syndrome also have a 13-fold increased risk of developing non-Hodgkin’s lymphoma, and a subset of patients may have large granular cell lymphocyte syndrome (Question #28 below). Some patients develop nodular regenerative hyperplasia of the liver with portal hypertension and varices that can bleed.
Treatment is the same as that for RA patients with joint disease and can include use of methotrexate or other DMARD therapy. With control of the RA, leukopenia may improve. Granulocyte colony-stimulating factor (G-CSF) has been used and shown effective at increasing WBC counts and decreasing infections in some patients (neutrophils <1000/mm3). However, G-CSF can be associated with increased arthritis and vasculitis in some Felty’s syndrome patients when the WBC count is raised. Splenectomy is reserved for patients with severe, recurrent bacterial infections or patients with chronic non-healing leg ulcers who are not responsive or tolerant to drug therapy. Unfortunately, neutropenia recurs in 25% of patients who undergo splenectomy.