Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prognosis of large granular lymphocyte proliferation with rheumatoid arthritis (pseudo-Felty's syndrome) remains uncertain. We report a case with a 15-year follow-up. To date, the patient has not developed lymphadenopathy, splenomegaly, abnormalities in erythrocyte or platelet counts, neutropenia or severe or unexplained infections. This favorable course is not ascribable to an unusual lymphocyte phenotype (CD3+, CD8+, CD57+). A beneficial effect of methotrexate therapy is possible.
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PMID:Pseudo-felty's syndrome. Report of a case with no symptoms for at least 15 years. 906 12

Felty's syndrome (FS) (rheumatoid arthritis with neutropenia and splenomegaly) has a poor prognosis, largely because of the high risk of severe infection. Granulocyte colony-stimulating factor (G-CSF) is an emerging treatment for chronic neutropenia. We prospectively monitored its use in eight patients with recurrent infections or who required joint surgery. Significant side-effects were documented in five, including nausea, malaise, generalized joint pains, and in one patient, a vasculitic skin rash. In two patients treatment had to be stopped, and in these cases G-CSF had been started at full vial dosage (300 micrograms/ml filgrastim or 263 micrograms/ml lenograstim) alternate days or daily. G-CSF treatment was continued in three patients by restarting at reduced dose, and changing the proprietary formulation. G-CSF raised the neutrophil count, reduced severe infection, and allowed surgery to be performed. A combined clinical and laboratory index suggested that long-term treatment (up to 3.5 years) did not exacerbate the arthritis. Once on established treatment, it may be possible to use smaller weekly doses of G-CSF to maintain the same clinical benefit. One of the three patients whose FS was associated with a large granular T-cell lymphocytosis showed a reduction in this subset of lymphocytes during G-CSF treatment.
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PMID:Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). 951 12

Felty's syndrome is a rare disorder characterized as a systemic manifestation of severe rheumatoid arthritis associated with granulocytopenia and splenomegaly. We report a retrospective analysis of a series of seven patients treated successfully with low-dose methotrexate. leading to sustained clinical improvement (number of swollen joints) and normalization of the granulocyte count for an observation period of 1 yr. Our cohort is the largest ever published with methotrexate treatment of this rare condition. Our results confirm earlier single case reports suggesting methotrexate to be the first-choice treatment nowadays in Felty's syndrome.
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PMID:Methotrexate treatment in Felty's syndrome. 973 84

The article consists in a discussion of neutropenia caused by large granular lymphocytes (LGLs), illustrated by a review of the literature and case reports of five patients with LGL syndrome and one patient whose clinical characteristics were more consistent with classic Felty's syndrome. Recent years have witnessed advances in our knowledge of clonal expansions of suppressor-type T-cells and their capacity to induce neutropenia. The phenotypes of such cells are CD3+, CD8+ and CD57+. The syndrome is often seen in patients with rheumatoid arthritis, and if they also manifest splenomegaly it may be confused with Felty's syndrome. Appropriate evaluation and treatment of the condition are also discussed, and an attempt made to clarify the confusing terminology.
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PMID:[LGL syndrome can imitate Felty's syndrome. The diagnosis can be established by a simple test]. 1038 81

An increasing number of studies suggest the importance of antibodies in the pathogenesis of most systemic and organ-specific autoimmune diseases, although there is considerable controversy over the precise role of the autoantibodies involved. In humans, a major obstacle to progress is the identification and cloning of the relevant autoantibodies and autoantigens. Here, an approach based on the sequential use of antibody phage display and antigen expression libraries is developed and applied to a donor suffering from rheumatoid arthritis (RA), splenomegaly, and peripheral destruction of neutrophils leading to neutropenia (Felty's syndrome). An antibody phage display library was constructed from bone marrow from the donor and a high-affinity human mAb, ANA15, selected by panning against fresh neutrophils and independently by panning against a fixed cell line. The antibody showed strong staining of neutrophils and a number of cell lines. Probing of a lambdagt11 expression library from an induced myelomonocytic cell line with the mAb ANA15 identified the eukaryotic elongation factor 1A-1 (eEF1A-1) as a novel autoantigen. The specificity of ANA15 was confirmed by reactivity with both purified and recombinant eEF1A-1. Screening of a large panel of sera revealed that 66% of patients with Felty's syndrome had elevated levels of anti-eEF1A-1 antibodies. The cloning of this antibody-antigen pair should permit rational evaluation of any pathogenicity resulting from the interaction and its significance in neutropenia.
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PMID:Cloning and expression of a novel human antibody-antigen pair associated with Felty's syndrome. 1092 29

Felty's syndrome (FS) is a rare complication (less than 1%) of rheumatoid arthritis (RA), with the clinical feature of splenomegaly and neutropenia. Approximately 10-40% of FS patients have an expansion of peripheral blood large granular lymphocytes (LGL). This cell population mainly consists of two subsets: cytotoxic T cells (CD8+, CD57+) and natural killer cells (CD3-,CD8-,CD56+). It has been hypothesised that LGL expansion could be responsible for neutropenia by suppressing neutrophil precursors in the bone marrow, but various mechanisms have been proposed to explain this association. We report a case of a 60-year-old woman with rheumatoid factor positive RA who developed LGL expansion responsible for splenomegaly, but without neutropenia. In conclusion, LGL expansion is an uncommon complication of RA and may be responsible for both FS and clinical pictures resembling FS.
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PMID:[Case report: rheumatoid arthritis and large granular lymphocytes syndrome]. 1256 71

Felty's syndrome is characterized by neutropenia, splenomegaly, and leg ulcers in patients with rheumatoid arthritis. The pathogenesis of the neutropenia is an immune-mediated process that involves immune complexes, antineutrophil antibodies, and abnormal white cell kinetics. We prescribed salazosulfapyridine to a 65-year-old woman with this syndrome. The neutropenia improved along with a reduction in neutrophil-bound IgG, demonstrated by flow cytometric analysis. Salazosulfapyridine may be of benefit for the treatment of Felty's syndrome, and flow cytometry can be used to monitor disease activity and therapeutic efficacy.
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PMID:Salazosulfapyridine-induced remission of Felty's syndrome along with significant reduction in neutrophil-bound immunoglobulin G. 1256 3

Felty's syndrome (FS) comprises a triad of rheumatoid arthritis (RA), neutropenia and splenomegaly, occurring in less than 1% of RA patients. Clinically it is characterized by severe joint destruction contrasting with moderate or absent joint inflammation and severe extra-articular disease, including a high frequency of rheumatoid nodules, lymphadenopathy, hepatopathy, vasculitis, leg ulcers, skin pigmentation etc. Recurrent bacterial infections are mostly due to the severe, otherwise unexplained neutropenia. The cause of neutropenia lies in both decreased granulopoiesis and increased peripheral destruction of granulocytes. Recurrent infections may lead to increased mortality. Spontaneous remission of the syndrome also occurs. Over 95% of FS patients are positive for rheumatoid factor (RF), 47-100% are positive for antinuclear antibody (ANA), and 78% of patients have the HLA-DR4*0401 antigen. Some 30% of FS patients have large granular lymphocyte (LGL) expansion. LGL expansion associated with uncomplicated RA is immunogenetically and phenotypically very similar to but clinically different from FS. Neutropenia of FS can be effectively treated with disease-modifying anti-rheumatic drugs (DMARDs), the widest experience being with methotrexate (MTX). Results of treatment with granulocyte colony-stimulating factor (G-CSF) are encouraging, but there is no experience with other biological agents. Splenectomy results in immediate improvement of neutropenia in 80% of the patients, but the rate of infection decreases to a lesser degree.
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PMID:Felty's syndrome. 1545 23

A 68-year-old woman with Felty's syndrome was admitted to our hospital due to breathlessness. She was diagnosed as having rheumatoid arthritis at age 59 years. Abdominal computed tomography indicated ascites, splenomegaly and liver atrophy. She had no antigens or antibodies for hepatitis virus, or antibodies for mitochondria with the exception of antinuclear antibody. According to the International Autoimmune Hepatitis (AIH) scoring system, she was diagnosed as having chronic hepatitis, compatible with AIH. The association of Felty's syndrome with AIH is very rare and the most difficult problem to overcome is whether or not steroid therapy is necessary in patients with Felty's syndrome complicated by AIH.
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PMID:Felty's syndrome with chronic hepatitis and compatible autoimmune hepatitis: a case presentation. 1589 48

The successful treatment of rheumatoid arthritis often requires the use of immunosuppressive medications. Although these agents have different potential toxicities, they share in common the potential for permitting the development of opportunistic infections. We describe 2 patients with chronic rheumatoid arthritis treated with immunosuppressive agents who developed progressive neutropenia with documented splenomegaly. The differential diagnosis included Felty's syndrome versus bone marrow suppression from their immunosuppressive medication. Although both patients had the triad of Felty's syndrome, high titer seropositive rheumatoid arthritis with splenomegaly and neutropenia, the diagnosis of this syndrome relies on excluding other potential causative factors. Further investigation revealed that both patients had disseminated histoplasmosis with bone marrow involvement, which most likely represented reactivation from prior exposure to Histoplasma capsulatum. Opportunistic infections such as disseminated histoplasmosis can mimic other disease processes, including Felty's syndrome, and are important to consider when there is a change in the clinical status of patients with rheumatic disease who are immunocompromised.
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PMID:Disseminated Histoplasmosis Mimicking Felty's Syndrome. 2283 3


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