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Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • Felty syndrome
  • Rheumatoid arthritis, splenomegaly and neutropenia
  • Familial Felty's syndrome



Felty's syndrome
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Felty's syndrome is a rare, potentially serious disorder that is defined by the presence of three conditions: rheumatoid arthritis (RA), an enlarged spleen (splenomegaly) and a decreased white blood cell count (neutropenia), which causes repeated infections.[1] Although some individuals with Felty's syndrome are asymptomatic, others can develop serious and life-threatening infections.[2] Symptoms of Felty's syndrome, in addition to those associated with the three conditions stated above, may include fatigue, fever, weight loss, discoloration of patches of skin,[1] mild hepatomegaly (enlarged liver), lymphadenopathy (swelling of lymph nodes), Sjögren syndrome, vasculitis, lower-extremity ulcers, and other findings.[2] The exact cause is unknown, but several risk factors have been proposed, including autoimmunity.[1][2] A few familial cases of the condition have been reported.[3] Treatment typically focuses on controlling the underlying RA; immunosuppressive therapy for RA may improve neutropenia and splenomegaly.[2]


References
  1. Felty Syndrome. NORD. December 31, 2010 Available at: http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Felty%20Syndrome. Accessed January 6, 2011.
  2. Richard M Keating. Felty syndrome. eMedicine. December 3, 2008 Available at: http://emedicine.medscape.com/article/329734-overview. Accessed January 6, 2011.
  3. Runge LA, Davey FR, Goldberg J, Boyd PR. The inheritance of Felty's syndrome in a family with several affected members. Journal of Rheumatology. February 1986;13(1):39-42. Available at: http://www.ncbi.nlm.nih.gov/pubmed/3701742. January 6, 2011.
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