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Author: Rebekah Condit, Texas A&M College
of Medicine, Class of 2012
Introduction:Felty's Syndrome (FS) is
classically defined as a triad of rheumatoid arthritis,
splenomegaly, and neutropenia, and is found in 1-3% of patients
with rheumatoid arthritis. First described in 1924 there are new
definitions and concurrent debate over therapy. There are no
randomized control trials on treatment of Felty's syndrome; current
goals of treatment focus on increasing the neutrophil count,
preventing infections, addressing the splenomegaly, and/or
treatment of rheumatoid arthritis.
Case Presentation: A 50-year-old Caucasian male
presents with a 2.5 day history of redness and increased swelling
of his right little finger. The symptoms began with a splinter, and
the next day he had erythema up to his right axilla, pain, fever,
chills, and clear fluid drainage. The patient has no other
significant history. The patient denied history of STDs, HIV, and
IVDA (IV drug abuse), but endorsed a 70-pack-year history of
tobacco abuse. On admission, the patient was afebrile with a
palpable spleen and erythema and ecchymosis of right little finger.
The patient's WBC count was 0.9 on two separate occasions, and
absolute neutrophil count was 108. His CRP and ESR were 117 and 29
respectively. The patient's workup revealed a positive ANA,
Rheumatoid factor, and anti-citrulline antibody along with clinical
and radiographic signs of rheumatoid arthritis. Splenomegaly
measuring 19 cm was confirmed on ultrasound. The triad of
rheumatoid arthritis, splenomegaly and neutropenia was consistent
with Felty's Syndrome. The patient was discharged on antibiotics, a
colony-stimulating factor for his neutropenia, and had follow-up
appointments with Rheumatology and Hematology clinics.
Discussion: Recent research suggests there is a
new definition for Felty's syndrome; unexplained neutropenia with
rheumatoid arthritis. The evolution of the disease has created a
debate about the classification and treatment of Felty's Syndrome.
Research has shown that Felty's syndrome may be included in a
spectrum of disease with T-cell large granular lymphocyte leukemia,
TLGL, with an umbrella of Rheumatoid arthritis. FS and TLGL are
clinically and biologically similar with no specific test for
differentiating the two diseases. This spectrum of disease leads to
a question of whether treatment of FS and TLGL should be early and
aggressive treatment of rheumatoid arthritis or treatment of
neutropenia, infection, and splenomegaly.
April 2012 Issue of IMpact