eMedicine Specialties > Rheumatology > Rheumatoid Arthritis

Felty Syndrome: Follow-up

Author: Richard M Keating, MD, FACR, FACP, Professor of Medicine, Co-Director, The University of Chicago Arthritis Center, Department of Medicine, Section of Rheumatology, The University of Chicago
Contributor Information and Disclosures

Updated: Dec 3, 2008

Follow-up

Further Inpatient Care

  • Admission is mandatory for patients with serious life-threatening infections. Patients need cultures and parenteral antibiotics.
  • Acknowledge the possibility of infection with encapsulated organisms because splenomegaly may be a marker for a dysfunctional reticuloendothelial system incapable of clearing these organisms. Staphylococcus aureus, streptococcal species, and gram-negative rods are potential infecting organisms.

Further Outpatient Care

  • Schedule patients with Felty syndrome for regular follow-up with a rheumatologist to monitor therapy and to assess progress.
  • No firm guidelines address immunization practice in patients with Felty syndrome, but ensuring vaccination against encapsulated organisms seems prudent.

Complications

  • Splenic rupture
  • Life-threatening infection
  • Toxicity due to immunosuppressive regimens
  • Portal hypertension and gastrointestinal bleeding due to nodular regenerative hyperplasia of the liver

Prognosis

  • Granulocytopenia is defined as an absolute neutrophil count (ANC) of less than 2000/µL, and the infection risk increases as the ANC drops. Infection incidence increases significantly when the polymorphonuclear leukocyte (PMN) count is less than 1000/µL.
  • Lymphoproliferative malignancies were more prevalent in a retrospective study of male patients with Felty syndrome treated at the Department of Veterans Affairs. In particular, the patients had an increased prevalence of non-Hodgkin lymphoma.9

Patient Education

  • Educate patients with Felty syndrome about the warning signs of infection and ensure that they have ready access to medical care.
  • Some practitioners supply patients with Felty syndrome with a broad-spectrum oral antibiotic to take at the first signs of a bacterial infection. Instruct patients that this is an individualized decision and advise them to contact their physician immediately if such a situation develops.

Miscellaneous

Medicolegal Pitfalls

  • Failure to acknowledge that many of the regimens used to treat rheumatoid arthritis (RA) can cause bone marrow suppression and neutropenia
  • Failure to diagnose Felty syndrome, thereby attributing the granulocytopenia to the RA treatment regimen
  • Failure to realize that active infection can transiently elevate the WBC count and mask the underlying condition
  • Failure to perform repeated examination of the abdomen in patients with RA, which is necessary to find early splenomegaly

Special Concerns

  • Neutropenia with LGL
    • This condition, often referred to as pseudo-Felty syndrome, 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 less than 1% of patients with RA. LGL usually develops at the onset of RA, while Felty syndrome 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 WBC count.
    • Thrombocytopenia, anemia, and splenomegaly may occur. In some situations, differentiation from Felty syndrome may require immunophenotype analysis.
    • Therapy for LGL is rarely necessary.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Kevin Kempf, MD, FACR, FACP, to the development and writing of this article.



More on Felty Syndrome

Overview: Felty Syndrome
Differential Diagnoses & Workup: Felty Syndrome
Treatment & Medication: Felty Syndrome
Follow-up: Felty Syndrome
References
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References

  1. Felty AR. Chronic arthritis in the adult associated with splenomegaly and leukopenia. Bull Johns Hopkins Hosp. 1924;35:16.

  2. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev. Sep 2006;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. Sep 2002;46(9):2384-91. [Medline].

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

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

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

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

  8. 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. Feb 2003;30(2):404-6. [Medline].

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

  10. Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol. Oct 2004;18(5):631-45. [Medline]. [Full Text].

  11. Barton JC, Prasthofer EF, Egan ML, et al. Rheumatoid arthritis associated with expanded populations of granular lymphocytes. Ann Intern Med. Mar 1986;104(3):314-23. [Medline].

  12. Breedveld FC, Fibbe WE, Hermans J, et al. Factors influencing the incidence of infections in Felty's syndrome. Arch Intern Med. May 1987;147(5):915-20. [Medline].

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

  14. Ellman MH. Leukocyte colony-stimulating factors for rheumatologists. J Clin Rheumatol. 1997;3(4):217-223.

  15. Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev. Sep 1996;10(3):177-84. [Medline].

  16. Rosenstein ED, Kramer N. Felty's and pseudo-Felty's syndromes. Semin Arthritis Rheum. Dec 1991;21(3):129-42. [Medline].

  17. Starkebaum G. Use of colony-stimulating factors in the treatment of neutropenia associated with collagen vascular disease. Curr Opin Hematol. May 1997;4(3):196-9. [Medline].

  18. Ward MM. Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001. Arthritis Rheum. Apr 2004;50(4):1122-31. [Medline].

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Further Reading

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Keywords

Felty syndrome, FS, Felty's syndrome, pseudo-Felty syndrome, pseudo-Felty's syndrome, rheumatoid arthritis, RA, splenomegaly, granulocytopenia, rheumatoid factor, RF, large granular lymphocytosis syndrome, LGL

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Contributor Information and Disclosures

Author

Richard M Keating, MD, FACR, FACP, Professor of Medicine, Co-Director, The University of Chicago Arthritis Center, Department of Medicine, Section of Rheumatology, The University of Chicago
Richard M Keating, MD, FACR, FACP is a member of the following medical societies: American College of Physicians and American College of Rheumatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; 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, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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