Felty's Syndrome consists of splenomegaly with neutropenia in patients with rheumatoid arthritis.
Primary features:
(1) rheumatoid arthritis: typically long-standing, seropositive, nodular and deforming with systemic involvement
(2) neutropenia
(3) splenomegaly: highly variable and may be mild, moderate or severe. A few patients may not have detectable splenomegaly.
Additional findings:
(1) anemia with reticulocytosis
(2) thrombocytopenia, usually mild
(3) lymphopenia
(4) weight loss
(5) lymphadenopathy
(6) leg ulcers
(7) skin hyperpigmentation
(8) peripheral neuropathy
(9) chronic, indolent infections
(10) nodular regenerative hyperplasia of the liver, which may be associated with portal hypertension
Associated findings:
(1) HLA-DR4 positive in > 90% (many homozygous for the HLA-Dw4 allele)
(2) high titers of rheumatoid factor
(3) high titer of antinuclear antibodies
(4) hypocomplementemia
