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At least 19 recordsLinked to original sources
PubMed1996

Treatment of the neutropenia of Felty syndrome.

This review sets out to synthesize and critically evaluate the current reported data regarding therapeutic options for the neutropenia associated with Felty syndrome (Felty neutropenia). A MEDLINE search and bibliographies from recent reviews were used to identify trials and case reports that provided sufficient data to evaluate the effect of various interventions on both the neutropenia and the clinical course of patients with Felty syndrome. Data were obtained on baseline hematologic profiles, bone-marrow biopsies, and patient characteristics; length of follow-up; hematologic and clinical responses to the various interventions; and side-effect profiles. Treatment with hemopoietic growth factors or methotrexate can produce sustained hematologic and clinical responses with an acceptable side-effect profile. Splenectomy produces a long-term hematologic response in 80% of patients. Patients who do not respond hematologically have a higher incidence of non-fatal infections, but a significant minority (46%) do not experience any infections; the incidence of fatal infections is 12%, regardless of whether a hematologic response occurs. Of the patients who had infections prior to surgery, 55% did not experience further infections after splenectomy. Initial treatment of Felty neutropenia should consist of hemopoietic growth factors because of their rapid onset of action and relatively low incidence of side-effects. Splenectomy is a reasonable option if growth factors are ineffective and rapid amelioration of neutropenia is needed. Methotrexate offers a potentially promising alternative for the treatment of both the rheumatologic and the hematologic manifestations of Felty syndrome.

Felty Syndrome↗
PubMed1996

An unusual association of Felty syndrome and TCR gamma delta lymphocytosis.

Felty syndrome, comprised of neutropenia, rheumatoid arthritis and splenomegaly, occurs in approximately 1% of patients with rheumatoid arthritis. Up to one third of these patients have an increased number of large granular lymphocytes. The usual immunophenotype of these cells is CD3+, CD8+, CD57+, T cell receptor (TCR) alpha beta. A patient with Felty syndrome and large granular lymphocytosis, who had an unusual immunophenotype CD3+, CD4-, CD8-, TCR gamma delta, is described. Her neutropenia responded to treatment with granulocyte colony stimulating factor (G-CSF), which was given in order to raise her neutrophil count prior to bilateral knee replacement surgery. Thus, Felty syndrome with large granular lymphocytosis is a heterogeneous condition, one in which TCR gamma delta large granular lymphocytosis may be found, and also shows a response to treatment with G-CSF.

Adult↗
PubMed1977

Improvement of rheumatoid arthritis following splenectomy for Felty syndrome.

A patient with Felty syndrome underwent splenectomy because of persistent high fever, leg ulcer, and granulocytopenia. Subsequently, his rheumatoid synovitis went into prolonged remission. In addition, the leg ulcer healed; his WBC count, hemoglobin level, temperature, ESR, and weight returned to normal; the rheumatoid factor titer dropped substantially; and the antinuclear antibodies disappeared. These changes have persisted to the present time, more than 5 1/2 years after surgery.

Adult↗
PubMed1994

Incidence of cancer among men with the Felty syndrome.

OBJECTIVE: To estimate the incidence of cancer (especially lymphoproliferative malignancies) in patients with the Felty syndrome. DESIGN: A retrospective cohort study. SETTING: A computerized database of all discharge records for 1969 to 1990 from a Veterans Affairs hospital. PATIENTS: 906 men with a discharge diagnosis of the Felty syndrome. MEASUREMENTS: Standardized incidence ratios (SIR) (ratios of observed-to-expected events) estimated the risk for specific cancers. Hospital records confirmed the diagnoses of the Felty syndrome and cancer. RESULTS: We observed a twofold increase in total cancer incidence (137 patients; SIR = 2.09; 95% CI, 1.8 to 2.5). The risk for non-Hodgkin lymphoma (19 patients; SIR = 12.8, CI, 7.7 to 20.0) was much greater than the twofold increase in risk for lymphoma generally reported for rheumatoid arthritis. The risk for leukemia was increased but only within 5 years of the first hospitalization for the Felty syndrome, (13 patients; SIR = 7.67; CI, 4.1 to 13.1). CONCLUSION: The increased risk for non-Hodgkin lymphoma after the Felty syndrome in our study is similar to the risk associated with the Sjögren syndrome and may reflect similar immunostimulatory mechanisms.

Adult↗
PubMed1986

Parenteral gold therapy in the Felty syndrome. Experience with 20 patients.

Most of the patients with the Felty syndrome suffer from such complications as fevers, infections, cutaneous ulcers, and vasculitis. Unfortunately, there are no therapeutic interventions that are predictably beneficial. We report our experience with 20 patients who received parenteral gold therapy for 2 to 114 months (mean, 23.6 months). All had complications of the Felty syndrome. On parenteral gold therapy, 60% had a complete response, 20% had a partial response, and 20% were unresponsive by preselected criteria. No serious complications were encountered. We think that parenteral gold therapy should be considered early, before other agents, in the treatment of this condition.

Adult↗
PubMed1990

The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations.

Thirty-two patients with the Felty syndrome, defined by the presence of rheumatoid arthritis, splenomegaly, and neutropenia, have been studied in comparison with 32 patients with rheumatoid arthritis matched for age, sex, and disease duration, and 9 patients with rheumatoid arthritis and idiopathic neutropenia. Patients with the Felty syndrome had severe destructive arthritis, which progressed during follow-up despite little evidence of objective synovitis, and a higher frequency of extra-articular manifestations, including vasculitis. Bacterial infection tended to occur in patients with the lowest neutrophil count but continued to occur in some despite normalization of the WBC. Prognosis was poor and 8 deaths occurred, predominantly from sepsis. Serologic features were prominent. High titers of IgG rheumatoid factor and circulating immune complexes characterized patients with persistent neutropenia. A family history of rheumatoid arthritis was more common in patients with the Felty syndrome. The association with HLA DR4 was very strong; in addition there was an increased frequency of the DQw3 variant, 3b, suggesting that HLA Class II genes in linkage with DR4 may contribute to disease expression.

Adult↗
PubMed1991

Felty's and pseudo-Felty's syndromes.

Felty's syndrome, consisting of rheumatoid arthritis, leukopenia, and splenomegaly, has been recognized as a distinct clinical entity for more than 60 years. Clinical and laboratory manifestations of the condition are reviewed. The major sources of morbidity and mortality remain recurrent local and systemic infections. Immunogenetic analysis shows a strong association with HLA-DR4, in addition to DQ beta 3b and C4B null allele. Potential mechanisms of neutropenia are contrasted, including impaired granulopoiesis and neutrophil-immune complex interactions. Lithium carbonate and splenectomy may have a role in the treatment of fulminant disease. Maintenance therapy should be directed at control of the underlying inflammatory arthropathy. A syndrome of proliferation of large granular lymphocytes and neutropenia, associated with rheumatoid arthritis in 23% to 39% of cases, has been described recently. Cases of "pseudo-Felty's" syndrome are often confused with traditional Felty's syndrome, which has twice the prevalence. The clinical and laboratory distinctions between these two conditions are elaborated.

Antigens, CD↗
PubMed1991

GM-CSF in the treatment of Felty syndrome.

Many therapeutic agents have been tried with variable success in the treatment of Felty neutropenia, but the reports are anecdotal. We now describe the second trial of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF), in a splenectomized, infected patient with Felty syndrome.

Felty Syndrome↗
PubMed1992

[Felty's syndrome].

Felty's syndrome is a variant of rheumatoid arthritis with a triad of leukopenia, splenomegaly and typical rheumatoid arthritis, and often accompanies the other extraarticular symptoms, such as subcutaneous nodules, lymphadenopathy, anemia, thrombocytopenia, leg ulcers, fever and weight loss. In addition to the decreased number of leukocyte, impaired functions of leukocytes were also revealed. These changes were considered as the cause of repeated infections, which was the major cause of death in Felty's syndrome. Splenectomy is not fully effective for leukopenia and for the repeated infection. Glucocorticoid therapy can increase leukocyte count, at least tentatively. It has been reported that gold was effective for leukopenia, repeated infection and skin ulcer. However, the definitive way of treatment of this syndrome based on the pathogenesis of this syndrome, remains to be established.

Adolescent↗
PubMed1982

Long-term outcome in Felty's syndrome.

Felty's syndrome has again been shown to be a severe form of systemic rheumatoid disease characterised by severe joint involvement, many extra-articular features, and a high incidence of infection. In addition we have shown that splenectomy was not protective for infections and in fact may on occasion contributed to infection. Furthermore, although most patients had an increase in white blood cell count after splenectomy, 50% of patients without splenectomy showed a similar increase in white blood cell counts at follow-up. Mortality in Felty's syndrome was high, with infection being the main cause of death.

Aged↗
PubMed1991

Strong primary selection for the Dw4 subtype of DR4 accounts for the HLA-DQw7 association with Felty's syndrome.

Felty's syndrome (FS) is a rare complication of rheumatoid arthritis (RA) previously shown to be strongly associated with HLA-DR4 and less significantly with HLA-DQw7. To map more precisely the HLA locus responsible for susceptibility to FS, we have examined HLA-DR4 and DQ beta-chain polymorphisms in FS patients and controls using restriction fragment length polymorphism analysis and polymerase chain reaction amplification in conjunction with oligonucleotide probing. The increased frequency of DR4 in FS (93% vs. 32% controls) was due almost entirely to enrichment for the Dw4 subtype (88% vs. 20% controls) with a secondary increase of the Dw14 subtype. Dw10 and Dw13 subtypes of DR4 were absent from the patient group. Increase in DQw7 frequency among DR4 FS patients could be accounted for by linkage disequilibrium between Dw4 and DQw7 alleles. Whereas susceptibility to RA is strongly associated with a conserved HLA-DR beta epitope associated with several DRB1 alleles, it is primarily the Dw4 allele which is associated with progression to Felty's syndrome. The finding that amino acid sequence variation at the DR4B1 locus rather than DQB1 is associated with development of FS will have important implications for the development of novel immunotherapies which are major histocompatibility complex allele-dependent.

Amino Acids↗
PubMed1985

Felty syndrome: autoimmune neutropenia or immune-complex-mediated disease?

Immunofluorescence on polymorphonuclear cells (PMN) of patients with Felty syndrome (FS) revealed increased amounts of IgG, IgA, and IgM bound to the PMN surface compared with PMN of patients with rheumatoid arthritis alone. A positive correlation was found between the score for surface-bound immunoglobulins on FS-PMN and the results of the Clq binding assay in FS sera. After preincubation with sera from 20 patients with FS, immunofluorescence on PMN from healthy controls (HC) showed that these cells had bound IgG, IgA, and IgM. However F(ab')2 fragments of IgG from FS sera did not bind to PMN, although the antigen-binding reactivity of the F(ab')2 fragments was maintained as shown by control experiments. Immunoglobulins eluted from FS-PMN failed to bind to HC-PMN, whereas the corresponding IgG of patients with autoimmune neutropenia was bound. Gel filtration of FS sera on Sepharose 4B showed that the binding of IgG in FS sera to PMN did not coincide with the 7S peak but occurred mainly in fractions containing larger material. No binding of IgA and IgM to HC-PMN was found after incubation with FS sera pretreated with polyethylene glycol (PEG) to precipitate immune complexes. These results indicate that in sera of patients with FS the PMN-binding reactivity of IgG, IgA, and IgM is due to the binding of immune complexes containing these immunoglobulins and not to presence of autoantibodies directed to antigens on the neutrophil surface.

Antigen-Antibody Complex↗
PubMed1991

[Felty's syndrome].

Felty's syndrome (FS) consists of the triad: rheumatoid arthritis (RA), leukopenia and splenomegaly. FS occurs in approximately 1% of patients with RA. In this syndrome, the risk of infection is increased and anaemia, thrombocytopenia and cutaneous ulcers are more frequently observed. The literature is reviewed on the basis of a case history. The pathogenesis is unknown but is probably multifactorial. Cell antibodies, increased occurrence of immune complexes, inhibited neutrophil production, altered neutrophil distribution and reduced neutrophil function have been observed. The main indication for treatment is present if the patient has severe neutropenia (less than 0.1 x 10(9)/l) and repeated infections. Various methods of treatment are available. The most important are: gold, low-dose methotrexate, lithium, methylprednisolone pulse therapy, penicillamine and splenectomy. According to the literature, conventional steroid treatment cannot be recommended.

Aged↗
PubMed1987

Comparison of T cell receptor gene rearrangements in patients with large granular T cell leukemia and Felty's syndrome.

Felty's syndrome (FS) refers to the occurrence of rheumatoid arthritis, splenomegaly, and neutropenia. A subset of these patients has recently been described with a chronic T cell leukemia of large granular lymphocytes (LGCL). To examine the spectrum of lymphocyte abnormalities in FS and LGCL, we examined phenotypic and genotypic properties of lymphocytes from eight FS patients. In two of these FS patients, we observed an elevated proportion of T cells with an unusual phenotype (CD3+/Leu-7+/Leu-8-/CR3+) (46 +/- 5% of mononuclear cells). The FS lymphocytes had large granular morphology on Wright-Giemsa stain and were active in antibody-dependent cellular cytotoxic activity. This phenotype, morphology, and activity was similar to LGCL patients except that the latter T cells additionally expressed the Fc-IgG receptor recognized by monoclonal antibody Leu-11 (CD 15). In the remaining six FS patients, the proportion of CD3+/Leu-7+/CR 3+ T cells was only 10 +/- 8%, which was not significantly different from age-matched normal subjects (6.6 +/- 2.2%). To determine the clonality of T lymphocytes in FS and LGCL, we examined DNA for rearrangements of the T cell antigen receptor beta-chain (Ti beta) and gamma-chain (Ti gamma) genes by using Southern blotting techniques. We found a clonal rearrangement of the Ti beta 1 and Ti gamma genes in both LGCL patients. In contrast, no clonal rearrangements of Ti beta or Ti gamma genes were detected in lymphocytes from the FS patients. These results indicate that FS patients are heterogeneous in their phenotype and that one subset exhibits polyclonal expansion of an unusual lymphocyte subset.

Antibody-Dependent Cell Cytotoxicity↗
PubMed1995

V gamma 9-V delta 2+ gamma delta T cells from a patient with Felty syndrome that exhibit aberrant response to triggering of the CD3 molecule can regulate immunoglobulin secretion by B cells.

Expanded populations of T lymphocytes bearing gamma delta T cell receptors have been detected in several patients with Felty syndrome. The goal of this study was to functionally characterize these lymphocytes in a newly described patient with this disease. For this, fluorescence-activated cell sorter analysis of T cell surface antigens, proliferation, and tumor necrosis factor alpha enzyme-linked immunosorbent assays, as well as quantitative assays of immunoglobulins secreted by pokeweed mitogen-driven B cells were performed. The gamma delta cells, that expressed a CD3+CD4-V gamma 9-V delta 2+C delta + phenotype, and constituted 60% of the peripheral blood T cells, did not proliferate after triggering with anti-CD3, but did secrete tumor necrosis factor alpha and the addition of these cells to pokeweed mitogen-stimulated B cells from the patient decreased their secretion of immunoglobulin M while augmenting IgG secretion. These data suggest that the expanded anergic V gamma 9-V delta 2+ gamma delta cells can play an immunoregulatory role in the patient.

Aged↗
PubMed1996

[Felty syndrome: a therapy-resistant variant of chronic rheumatoid arthritis? 2 case reports and literature review].

Felty's syndrome is a rare but serious extra-articular manifestation of rheumatoid arthritis. Morbidity as well as mortality are increased on account of greater susceptibility to infectious agents. We report on two patients suffering from Felty's syndrome who were successfully treated by cyclophosphamide. A review of the literature with special regard to treatment of Felty's syndrome is given.

Adult↗
PubMed1995

[Rheumatoid arthritis, neutropenia and splenomegaly: the Felty syndrome].

HISTORY AND FINDINGS: A 59-year-old asymptomatic man, first diagnosed to have rheumatoid arthritis 27 years ago, was admitted to hospital because of splenomegaly and neutropenia, first noted 2 years ago. Physical examination confirmed splenomegaly and also revealed pretibial hyperpigmentation, but no evidence of active rheumatoid arthritis. EXAMINATIONS: Biochemical tests showed relative and absolute neutropenia (white blood cell count 2200/microliters; 1% neutrophils), thrombocytopenia and polyclonal hypergammaglobulinaemia. He also had increased erythrocyte sedimentation rate (38/92), a high titre of rheumatic factor (2128 IU/ml) and increased circulating immune-complexes (74%). Thoracic and abdominal computed tomography provided no evidence of malignant tumor. The spleen measured 15 x 7 x 10 cm. Bone-marrow biopsy from the iliac crest revealed abnormal maturation of granulopoiesis and marked lymphoid infiltration. The clinical triad of rheumatoid arthritis, splenomegaly and neutropenia are diagnostic of Felty's syndrome. As the patient was asymptomatic there was no indication for treatment. CONCLUSION: Felty's syndrome is a rare condition demanding considerable effort in differential diagnosis.

Biopsy↗