Indications for splenectomy in 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 (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".)
RATIONALE FOR SPLENECTOMY
Splenectomy has been an important component of therapy in Felty’s syndrome (FS) for many years . 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 . (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 . However, subsequent studies suggest that other factors play a more significant role in the pathogenesis of granulocytopenia . Regardless of the mechanism, splenectomy leads to an increase in granulocytes in the peripheral blood within minutes or hours in most cases .
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