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Felty Syndrome Differential Diagnoses

  • Author: Richard M Keating, MD, FACR, FACP; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Feb 12, 2014
 
 

Diagnostic Considerations

In addition to the conditions listed in the differential diagnosis, other problems to be considered include the following:

  • Chronic infection
  • Drug reactions
  • Other rheumatologic diseases
  • Infiltrative diseases
  • HIV infection
  • Neutropenia with large granular lymphocytosis (LGL), also known as pseudo-Felty syndrome

The last of these is a particular concern. LGL is a chronic leukemia characterized by a clonal expansion of cytotoxic T cells bearing the CD16 and CD57 markers. The large granular lymphocytes may occur in both the periphery and the bone marrow. LGL is an unusual entity that occurs in fewer than 1% of patients with RA. It usually develops at the onset of RA, whereas FS occurs later in the disease course. The sex incidence is equal, and the severity of the underlying RA is less marked.

Neutropenia may occur in conjunction with a normal or increased total white blood cell (WBC) count. Thrombocytopenia, anemia, and splenomegaly may occur. In some situations, differentiation from FS may require immunophenotype analysis. Therapy for LGL is rarely necessary.

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Richard M Keating, MD, FACR, FACP Program Director, Rheumatology Fellowship, Scripps Clinic

Richard M Keating, MD, FACR, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Kavitta B Allem, MD Fellow in Rheumatology, Scripps Clinic

Disclosure: Nothing to disclose.

Dana A Copeland, MD Department of Rheumatology, Scripps Clinic

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

References
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  2. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev. 2006 Sep. 20(5):245-66. [Medline].

  3. Hellmich B, Csernok E, Schatz H, et al. Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. Arthritis Rheum. 2002 Sep. 46(9):2384-91. [Medline].

  4. Campion G, Maddison PJ, Goulding N, James I, Ahern MJ, Watt I, et al. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine (Baltimore). 1990 Mar. 69(2):69-80. [Medline].

  5. Gridley G, Klippel JH, Hoover RN, et al. Incidence of cancer among men with the Felty syndrome. Ann Intern Med. 1994 Jan 1. 120(1):35-9. [Medline].

  6. Brücker R, Schlumpf U. [Felty syndrome: a therapy-resistant variant of chronic rheumatoid arthritis? 2 case reports and literature review]. Praxis (Bern 1994). 1996 Apr 16. 85(16):534-40. [Medline].

  7. Ghavami A, Genevay S, Fulpius T, et al. Etanercept in treatment of Felty's syndrome. Ann Rheum Dis. 2005 Jul. 64(7):1090-1. [Medline].

  8. Talip F, Walker N, Khan W, et al. Treatment of Felty's syndrome with leflunomide. J Rheumatol. 2001 Apr. 28(4):868-70. [Medline].

  9. Ishikawa K, Tsukada Y, Tamura S, et al. Salazosulfapyridine-induced remission of Felty's syndrome along with significant reduction in neutrophil-bound immunoglobulin G. J Rheumatol. 2003 Feb. 30(2):404-6. [Medline].

  10. Sordet C, Gottenberg JE, Hellmich B, et al. Lack of efficacy of rituximab in Felty's syndrome. Ann Rheum Dis. 2005 Feb. 64(2):332-3. [Medline].

  11. Chandra PA, Margulis Y, Schiff C. Rituximab is useful in the treatment of Felty's syndrome. Am J Ther. 2008 Jul-Aug. 15(4):321-2. [Medline].

  12. Weinreb N, Rabinowitz A, Dellaripa PF. Beneficial response to rituximab in refractory Felty Syndrome. J Clin Rheumatol. 2006 Feb. 12(1):48. [Medline].

  13. Tomi AL, Lioté F, Ea HK. One case of Felty's syndrome efficiently treated with rituximab. Joint Bone Spine. 2012 Mar 6. [Medline].

  14. Narváez J, Domingo-Domenech E, Gómez-Vaquero C, López-Vives L, Estrada P, Aparicio M, et al. Biological agents in the management of Felty's syndrome: a systematic review. Semin Arthritis Rheum. 2012 Apr. 41(5):658-68. [Medline].

 
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