Role of splenectomy for Felty's syndrome
- Jonathan Kay, MD
Jonathan Kay, MD
- Timothy S. and Elaine L. Peterson Chair in Rheumatology
- Professor of Medicine and Director of Clinical Research, Rheumatology
- University of Massachusetts Medical School
- Section Editor
- Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
Ravinder N Maini, BA, MB BChir, FRCP, FMedSci, FRS
- Section Editor — Rheumatoid Arthritis
- Emeritus Professor of Rheumatology, Imperial College London
- Visiting Professor, Oxford University
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 . 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".)
GOALS OF THERAPY
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".)
INDICATIONS FOR SPLENECTOMY
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.
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- GOALS OF THERAPY
- INDICATIONS FOR SPLENECTOMY
- PERIOPERATIVE INFECTION RISK REDUCTION
- Role of immunization
- Preoperative granulocyte-colony stimulating factor therapy
- EFFICACY FOR NEUTROPENIA AND INFECTION
- OTHER BENEFITS OF SPLENECTOMY
- Thrombocytopenia and anemia
- Nonhematologic changes
- LARGE GRANULAR LYMPHOCYTE LEUKEMIA
- SUMMARY AND RECOMMENDATIONS