Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF)
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A combined clinical and laboratory index suggested that long-term treatment with G-CSF did not exacerbate the arthritis, and 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.Abstract:
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.read more
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Samter's Immunologic Diseases
TL;DR: This is the fifth edition of a comprehensive textbook on the immune system and its mechanisms and the book has been revised and reorganized throughout to reflect the changing face of immunology.
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References
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Clonal Diseases of Large Granular Lymphocytes
TL;DR: Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL, and X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome.
Journal ArticleDOI
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TL;DR: This chapter summarises studies performed principally by the own group over the last 9 years or so, but also by others in the field who have investigated the expression of cytokines in RA.
Journal ArticleDOI
Felty's and pseudo-Felty's syndromes.
Elliot D. Rosenstein,Neil Kramer +1 more
TL;DR: Clinical and laboratory manifestations of Felty's syndrome are reviewed, including impaired granulopoiesis and neutrophil-immune complex interactions, and potential mechanisms of neutropenia are contrasted.
Journal ArticleDOI
Treatment of the neutropenia of Felty syndrome
TL;DR: Hemopoietic growth factors or methotrexate offers a potentially promising alternative for the treatment of both the rheumatologic and the hematologic manifestations of Felty syndrome.
Journal ArticleDOI
Long-term outcome in Felty's syndrome
Carter Thorne,Murray B. Urowitz +1 more
TL;DR: It is shown that splenectomy was not protective for infections and in fact may on occasion contributed to infection, and although most patients had an increase in white blood cell count aftersplenectomy, 50% of patients withoutSplenectomy showed a similar increase inwhite blood cell counts at follow-up.