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Indications for splenectomy in 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 (RA) complicated by granulocytopenia and splenomegaly. The granulocytopenia in FS may improve when RA 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.

The goal in the treatment of granulocytopenia in FS is to prevent recurrent infections and to facilitate the resolution of ongoing bacterial infections. This may be accomplished by interventions that raise the granulocyte count, including the use of DMARDs, growth factors, other medications, and by splenectomy, although infections may also result from granulocyte dysfunction and other disease-related factors [1-4].

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 elsewhere. (See "Clinical manifestations and diagnosis of Felty's syndrome" and "Drug therapy in Felty's syndrome".)


Splenectomy has been an important component of therapy in Felty’s syndrome (FS) for many years [5]. The enlarged spleen in this disorder appears to play an important pathogenetic role in the development of granulocytopenia. How this occurs is incompletely understood, as both granulocyte sequestration within the spleen and production of antibodies or other factors that inhibit granulopoiesis may contribute [5]. (See "Clinical manifestations and diagnosis of Felty's syndrome".)

The old observation that splenic arterial blood contains a far greater number of granulocytes than splenic venous blood is compatible with an important role for sequestration [6]. However, subsequent studies suggest that other factors play a more significant role in the pathogenesis of granulocytopenia [7]. Regardless of the mechanism, splenectomy leads to an increase in granulocytes in the peripheral blood within minutes or hours in most cases [8].


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Literature review current through: Nov 2016. | This topic last updated: Mon Nov 30 00:00:00 GMT+00:00 2015.
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  1. Laszlo J, Jones R, Silberman HR, Banks PM. Splenectomy for Felty's syndrome. Clinicopathological study of 27 patients. Arch Intern Med 1978; 138:597.
  2. Ruderman M, Miller LM, Pinals RS. Clinical and serologic observations on 27 patients with Felty's syndrome. Arthritis Rheum 1968; 11:377.
  3. Breedveld FC, Fibbe WE, Cats A. Neutropenia and infections in Felty's syndrome. Br J Rheumatol 1988; 27:191.
  4. Dillon AM, Luthra HS, Conn DL, Ferguson RH. Parenteral gold therapy in the Felty syndrome. Experience with 20 patients. Medicine (Baltimore) 1986; 65:107.
  5. Goldberg J, Pinals RS. Felty syndrome. Semin Arthritis Rheum 1980; 10:52.
  6. 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.
  7. Burks EJ, Loughran TP Jr. Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. Blood Rev 2006; 20:245.
  8. DE GRUCHY GC, LANGLEY GR. Felty's syndrome. Australas Ann Med 1961; 10:292.
  9. Ward MM. Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001. Arthritis Rheum 2004; 50:1122.
  10. Breedveld FC, Fibbe WE, Hermans J, et al. Factors influencing the incidence of infections in Felty's syndrome. Arch Intern Med 1987; 147:915.
  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. 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) 1990; 69:69.
  13. 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.
  14. Khan MA, Kushner I. Improvement of rheumatoid arthritis following splenectomy for Felty syndrome. JAMA 1977; 237:1116.
  15. 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.
  16. Loughran TP Jr, Starkebaum G, Clark E, et al. Evaluation of splenectomy in large granular lymphocyte leukaemia. Br J Haematol 1987; 67:135.
  17. Rosen M, Brody F, Walsh RM, et al. Outcome of laparoscopic splenectomy based on hematologic indication. Surg Endosc 2002; 16:272.
  18. Nakamura H, Ohishi A, Asano K, et al. Partial splenic embolization for Felty's syndrome: a 10-year followup. J Rheumatol 1994; 21:1964.