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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and laboratory features of 72 patients with Felty's syndrome described within the last ten years have been compared with Felty's five original patients. Felty's syndrome appears to be a variant of rheumatoid arthritis with extra-articular manifestations in which leukopenia (usually due to neutropenia) and splenomegaly occur, although not always at the same time. Both are manifestations of the underlying disease process and are not necessarily otherwise related. The mechanism of the leukopenia is complex and abnormalities in leukocyte function appear to be as important as the leukopenia in predisposing patients with Felty's syndrome to infection. Functional abnormalities of the leukocytes in this syndrome are due in part to immune complex formation. Hypocomplementemia associated with this process may be another cause for the increased susceptibility to infection. It is proposed, therefore, that therapy in Felty's syndrome be directed at the underlying disease process, and gold salts and penicillamine should be considered for this purpose. Splenectomy should be reserved for specific situations, such as hemolytic anemia, severe thrombocytopenia, leg ulcers, and infections associated with profound leukopenia that are not responsive to medical therapy.
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PMID:Felty's syndrome: an analytical review. 33 Sep 14

The spectrum of disease was assessed in 43 patients with Felty's syndrome. Twenty-three underwent splenectomy. Although complete remission of neutropenia occurred in only two nonoperated patients, most of the patients had no serious infections during the subsequent period of observation. Operative morbidity was minimal in the splenectomized patients. On the basis of natural history of this condition, splenectomy should probably be restricted to neutropenic patients with serious or recurrent infections, patients with severe anemia requiring transfusions, those with the rare circumstance of profound thrombocytopenia, and those with non-healing leg ulcers.
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PMID:Felty's syndrome: when is splenectomy indicated? 397 Mar 27

Only limited data are reported on the response to granulocyte-colony stimulating factor (G-CSF) of large granular lymphocytosis-associated neutropenia. We report features of such a case who developed a suppurating leg ulcer following a dog bite. G-CSF was used to increase the neutrophil count, allowing the ulcer to be successfully skin grafted.
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PMID:Response of T-beta CD8+ lymphocytosis-associated neutropenia to G-CSF. 752 25

The cytokine granulocyte-macrophage colony stimulating factor (GM-CSF) has a pleiotropic activity that influences not only proliferation and differentiation of stem cells but also regulates several other cells involved in acute and chronic inflammation, as well as in the immune response. During the past 5 years, investigations have been performed to treat pathological situations rather than cytostatic induced neutropenia. This review presents the available experience on healing cut-, burn-wounds, leg ulcers, skin-graft resolution as well as cytostatic and radiotherapy induced mucositis by administration of GM-CSF. The next 5 years will probably be spent analysing the use of GM-CSF in the therapeutic dimension, considering the advantages that patients and clinicians can achieve. In particular, GM-CSF will have a greater role, when doses and mode of application are finally defined, in treating complicated cut-burn wounds and unsuccessful skin-grafted resolutions. The evidence reported by ongoing randomised clinical trials will benefit patients with severe chemotherapy and radiotherapy induced mucositis.
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PMID:New clinical applications of granulocyte-macrophage colony-stimulating factor. 910 73

We report the case of a boy with hereditary spherocytosis who presented with mild microcytic hypochromic anemia and recurrent leg ulcers that had been present since childhood. Chronic natural killer (NK) cell and B-cell lymphocytosis was detected 1 year after therapeutic splenectomy during investigation of recurrent episodes of neutropenia and persistent lymphocytosis. NK cells proved to be abnormal at immunophenotyping studies, and B-cells were polyclonal and displayed a normal immunophenotype. Genotypic analysis of T-cell receptor (TCR)-beta and TCR-gamma genes showed a germ-line pattern. The clinical course of this patient was characterized by multiple pulmonary infections and amygdalitis. We discuss the potential roles of persistent immune stimulation due to chronic hemolysis and severe leg ulcers and of splenectomy in the origin of NK cell lymphocytosis and the relationship between NK cells and recurrent infections, relapsing neutropenia, and polyclonal B-cell response.
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PMID:Abnormal NK cell lymphocytosis detected after splenectomy: association with repeated infections, relapsing neutropenia, and persistent polyclonal B-cell proliferation. 1209 47

We report a case of CD8(+)/V beta 5.1(+) T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3(+)/TCR alpha beta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-V beta 5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V beta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease.
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PMID:Cd8(+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin. 1211 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

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56(-) natural killer-cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56(-) LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.
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PMID:Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. 1996 15