Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Role of splenectomy for Felty's syndrome

Jonathan Kay, MD
Section Editor
Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
Deputy Editor
Paul L Romain, MD


Felty’s syndrome (FS) is an uncommon but severe subset of seropositive rheumatoid arthritis complicated by granulocytopenia and splenomegaly. The granulocytopenia in FS may improve when rheumatoid arthritis is treated with disease-modifying antirheumatic drugs (DMARDs), presumably because these drugs reverse underlying abnormalities in the immune and reticuloendothelial systems. Other therapies for FS increase circulating neutrophils by mechanisms which are not disease-specific.

Surgical intervention with splenectomy can result in improvement in the neutropenia and a reduction in infections in many patients with FS and has been performed for this purpose since the 1930s, although some patients do not respond to this therapy [1]. The need for splenectomy has decreased with the availability of medications that are often effective and because of the reduced incidence of the disorder; however, it remains a useful treatment option in patients unresponsive to medical therapies.

This topic will discuss the role of splenectomy in the treatment of FS. The clinical manifestations and diagnosis of FS and drug therapy for this disorder are reviewed separately. (See "Clinical manifestations and diagnosis of Felty's syndrome" and "Drug therapy in Felty's syndrome".)


The treatment goal in Felty's syndrome (FS) is reversal of the granulocytopenia to prevent recurrent and severe infections and febrile episodes, and to facilitate the resolution of ongoing bacterial infections. This may be accomplished by interventions that raise the granulocyte count, including the use of nonbiologic and biologic disease-modifying antirheumatic drugs (DMARDs), growth factors, and other medications, and by splenectomy, although infections may also result from granulocyte dysfunction and other disease-related factors [2-8]. (See "Drug therapy in Felty's syndrome".)


Splenectomy is generally indicated in the patient with Felty’s syndrome (FS) with recurrent or severe infections despite medical therapies; such patients usually have severe granulocytopenia (<1000 cells/microL). Prophylactic splenectomy is not justified in patients who do not become infected, even in the presence of marked granulocytopenia. The decision to perform splenectomy may also be influenced by the frequency and severity of infections, the degree and nature of comorbid conditions, and the patient’s overall medical status.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Jan 17, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev 1996; 10:177.
  2. Laszlo J, Jones R, Silberman HR, Banks PM. Splenectomy for Felty's syndrome. Clinicopathological study of 27 patients. Arch Intern Med 1978; 138:597.
  3. Ruderman M, Miller LM, Pinals RS. Clinical and serologic observations on 27 patients with Felty's syndrome. Arthritis Rheum 1968; 11:377.
  4. Breedveld FC, Fibbe WE, Cats A. Neutropenia and infections in Felty's syndrome. Br J Rheumatol 1988; 27:191.
  5. Dillon AM, Luthra HS, Conn DL, Ferguson RH. Parenteral gold therapy in the Felty syndrome. Experience with 20 patients. Medicine (Baltimore) 1986; 65:107.
  6. Narváez J, Domingo-Domenech E, Gómez-Vaquero C, et al. Biological agents in the management of Felty's syndrome: a systematic review. Semin Arthritis Rheum 2012; 41:658.
  7. Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol 2004; 18:631.
  8. Newman KA, Akhtari M. Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus. Autoimmun Rev 2011; 10:432.
  9. Breedveld FC, Fibbe WE, Hermans J, et al. Factors influencing the incidence of infections in Felty's syndrome. Arch Intern Med 1987; 147:915.
  10. Ward MM. Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001. Arthritis Rheum 2004; 50:1122.
  11. Sibley JT, Haga M, Visram DA, Mitchell DM. The clinical course of Felty's syndrome compared to matched controls. J Rheumatol 1991; 18:1163.
  12. Stanworth SJ, Bhavnani M, Chattopadhya C, et al. Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). QJM 1998; 91:49.
  13. Goldberg J, Pinals RS. Felty syndrome. Semin Arthritis Rheum 1980; 10:52.
  14. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev 2006; 20:245.
  15. DE GRUCHY GC, LANGLEY GR. Felty's syndrome. Australas Ann Med 1961; 10:292.
  16. WRIGHT CS, DOAN CA, BOURONCLE BA, ZOLLINGER RM. Direct splenic arterial and venous blood studies in the hypersplenic syndromes before and after epinephrine. Blood 1951; 6:195.
  17. Breedveld FC, van den Barselaar MT, Leigh PC, et al. Phagocytosis and intracellular killing by polymorphonuclear cells from patients with rheumatoid arthritis and Felty's syndrome. Arthritis Rheum 1985; 28:395.
  18. Khan MA, Kushner I. Improvement of rheumatoid arthritis following splenectomy for Felty syndrome. JAMA 1977; 237:1116.
  19. Stock H, Kadry Z, Smith JP. Surgical management of portal hypertension in Felty's syndrome: A case report and literature review. J Hepatol 2009; 50:831.
  20. Rosen M, Brody F, Walsh RM, et al. Outcome of laparoscopic splenectomy based on hematologic indication. Surg Endosc 2002; 16:272.