Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with classic rheumatoid arthritis developed severe neutropenia and increased numbers of large granular lymphocytes in the blood and bone marrow. These lymphocytes exhibited homogeneous surface membrane immunophenotypes of Leu5+, Leu11-, Leu4+, Leu3-, Leu2-, Leu7+ and Leu5+, Leu11+, Leu4+, Leu3-, Leu2+, Leu7-, respectively. In both patients, neutropenia was initially corrected with corticosteroid therapy; long-term improvement followed low-dose oral cyclophosphamide and methotrexate therapies. In these 2 patients and 12 previous patients with rheumatoid arthritis associated with expanded populations of immunophenotypically homogeneous large granular lymphocytes, neutropenia occurred in all 14, thrombocytopenia in 6, anemia in 7, and mild or moderate splenomegaly in 12. In contrast to Felty's syndrome, granular lymphocyte expansions in rheumatoid arthritis usually occur in older patients, may appear simultaneously with arthritis, and are usually associated with normal or elevated blood leukocyte counts. Mild hemocytopenias in these patients can often be managed with observation. Therapy with corticosteroids or immunosuppressive-cytotoxic drugs may be beneficial in more severe cases, but splenectomy is not recommended.
 ...
PMID:Rheumatoid arthritis associated with expanded populations of granular lymphocytes. 394 76

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.
 ...
PMID:Felty's syndrome: when is splenectomy indicated? 397 Mar 27

Five patients with polyarthritis and neutropenia had numerous circulating large granular lymphocytes with a phenotype attributed to immature natural killer cells. All five had splenomegaly and recurrent infections. Arthritis was most prominent at the wrists and hands, and all patients were considered to have atypical cases of Felty's syndrome. Antinuclear antibodies, rheumatoid factor, antineutrophil antibodies, and immune complexes were detected in most patients. Bone marrow biopsies revealed a maturation arrest at the myelocyte stage and lymphoid infiltrates. Large lymphocytes with azurophilic cytoplasmic granules were found on peripheral blood smears and showed a characteristic reactivity pattern with monoclonal antibodies suggesting a natural killer cell lineage. Peripheral blood mononuclear cells showed less than normal natural killer activity against K562 target cells. Increased numbers of large granular lymphocytes with a phenotype of immature natural killer cells may be important in the pathogenesis of neutropenia, humoral immune disturbances, and synovitis in a subset of patients with Felty's syndrome.
 ...
PMID:Polyarthritis and neutropenia associated with circulating large granular lymphocytes. 402 84

High dose intravenous gamma globulin (IV-IgG) was given to 5 patients with Felty's syndrome. The neutrophil count did not change with IV-IgG therapy and no side effects were encountered. We conclude that neutropenia in Felty's syndrome is not comparable to autoimmune hemocytopenia with respect to the response to IV-IgG.
 ...
PMID:High dose intravenous gamma globulin for Felty's syndrome. 405 90

We report our experience with 8 patients with Felty's syndrome who were treated with D-penicillamine for a mean of one year. Six of the 8 patients experienced improvement in their neutropenia. Cutaneous ulcers healed in 4 of 6, while recurrent infections cleared in 3 of 5 patients. The drug was withdrawn in 6 patients--lack of response in one, thrombocytopenia in one, urticaria in one, rash in one, and granulocytopenia in 2. One of the latter 2 patients developed pancytopenia and died. Although D-penicillamine is effective in treatment of Felty's syndrome, its side effects can be serious and potentially lethal. Its use should be limited to patients who have failed other treatments.
 ...
PMID:D-penicillamine in Felty's syndrome. 405 91

Twenty-seven patients with Felty's syndrome were studied by the colony forming unit in culture (CFU-C) assay for possible immune mechanisms within the bone marrow compartment that could contribute to the neutropenia. Depletion of bone marrow suppressor T cells resulted in normal CFU-C numbers in 7 patients. In 5 patients serum antiprecursor cell activity was detected. Blood monocytes failed to generate colony stimulating factor in 5 patients. Bone marrow-directed immune mechanisms are heterogeneous and could play a role in the pathogenesis of neutropenia in some patients with Felty's syndrome.
 ...
PMID:Heterogeneity of bone marrow-directed immune mechanisms in the pathogenesis of neutropenia of Felty's syndrome. 622 91

The authors report a case of Felty's syndrome in which are discussed over the data of literature: clinical features, hematological characteristics with a study of neutropenia mechanism and therapeutic possibilities.
 ...
PMID:[Felty's syndrome. A case report. Review of literature (author's transl)]. 626 42

The ability of sera from patients with systemic lupus erythematosus (SLE) and Felty's syndrome to induce increased adhesiveness of normal human neutrophils (PMN) was investigated. PMN from normal healthy donors were incubated in sera from 19 patients with active SLE, 12 with inactive SLE, 20 with Felty's, 24 with rheumatoid arthritis, and 34 normal persons. After incubation, the degree of adherence of the PMN to human endothelial cells in culture, their aggregation, and superoxide (O2-) generation were determined. Sera from patients with both active SLE and Felty's syndrome induced significantly increased PMN adherence to endothelial cells and PMN aggregation in vitro, compared with normal sera. This increased adherence to endothelial cells was maintained after heat treatment (56 degrees C for 30 minutes) of the sera. In O2- generation experiments, sera from patients with active SLE induced significantly increased O2- release from normal PMN using both fresh and heat-treated sera. Sera from Felty's patients demonstrated the same effect with heat-treated sera but not ith fresh sera. When sera from patients with active SLE and Felty's syndrome were used, all three parameters correlated significantly with each other in individual patients. In contrast, sera from the 12 patients with inactive SLE and 24 rheumatoid arthritis patients without Felty's failed to induce significant differences in the three parameters studied when compared with 34 normal controls. Fractionation of 3 SLE sera and 1 Felty's serum on Sephadex G-200 demonstrated that the adherence enhancing factor was present in both IgG and IgG-excluded fractions. The observed increased adhesiveness of PMN induced by SLE and Felty's sera may, at least in part, contribute to the neutropenia which is common in these diseases. Increased O2- release associated with PMN adherence may contribute to endothelial cell damage and vascular injury, which is also a common manifestation of these diseases.
 ...
PMID:Increased endothelial cell adherence, aggregation, and superoxide generation by neutrophils incubated in systemic lupus erythematosus and Felty's syndrome sera. 629 13

Sera from patients with Felty's syndrome, rheumatoid arthritis (RA) and controls were investigated for the presence of immune complexes (IC) using phagocytosis by normal polymorphonuclear leukocytes and direct immunofluorescence technique. IC visible as large cytoplasmic inclusions were seen in 19 of 24 cases of Felty's syndrome, 3 of 16 cases of RA, and all 3 patients with extraarticular manifestations, and none of 21 control sera. IC containing IgG, IgA and complement C3 were found in nearly all positive cases. IgM IC were found in only 8 of the Felty's syndrome cases, IgE in 5 and beta-2-microglobulin in one case, respectively. A tendency to increasing number of large inclusion positive cells in vitro was found inversely correlated to the number of circulating leukocytes in the Felty patients at the time of serum sampling. In contrast, small cytoplasmic inclusions were found both in Felty's syndrome and RA patients and in some of the controls, and IgG and C3 were the most frequent constituents in these cases. As these inclusions were found in all groups it may have little significance. IgE IC as determined by a PEG precipitation technique were positive in the same 5 cases of Felty's syndrome with IgE containing inclusions, and in one case of RA with extraarticular manifestations. The complexed IgE amounted to about 3% of the total serum concentration of IgE. Phagocytosed IC may be involved in the pathogenesis of neutropenia and contribute to the inflammatory processes in Felty's syndrome.
 ...
PMID:Phagocytosis by normal polymorphonuclear leukocytes of immune complexes from serum of patients with Felty's syndrome and rheumatoid arthritis with special reference to IgE immune complexes. 632 37

Twenty-one patients are described with a proliferation of morphologically mature T lymphocytes. The clinical course was chronic in most, and splenic enlargement the main clinical finding; skin involvement and lymphadenopathy were rare. The mean lymphocyte count at presentation was 8 X 10(9)/1 (range 0.75-24 X 10(9)/1). Nineteen of these patients showed some form of cytopenia (18 neutropenia, two red cell aplasia, eight thrombocytopenia) and one had hypogammaglobulinaemia. Seven patients had long-standing arthropathy serologically proven to be rheumatoid arthritis and these had previously been considered to have Felty's syndrome. Five of the group have died (three with an aggressive course), but most have remained stable for prolonged periods with a slow increase in peripheral lymphocyte count and marrow infiltration. Spontaneous regression was never observed but in two patients a prolonged remission was achieved by chemotherapy. The lymphocytes were morphologically and phenotypically homogeneous at presentation and remained so post-splenectomy; they contained azurophilic granules, stained with acid phosphatase but weakly or not at all with alpha napthyl acetate esterase. Membrane phenotyping shows the majority of the cells to be E+, Fc gamma+, OKT3+, OKT8+. Most cells do not stain with OKT1-like reagents and a significant number express HLA-Dr. From these and other reported cases it is clear that this condition represents a distinct entity resulting from the expansion of a subset of cytotoxic/suppressor T cells--the question of the benign or neoplastic nature of the disease remains open. Using T cell-specific antisera and E-rosetting techniques, a small percentage of CLL cases have been shown to be of T-cell origin (TCLL) (Dickler et al, 1973; Lille et al, 1973). Estimates of the percentage vary but in most series T-CLL has been diagnosed in less than 5% (Brouet & Seligmann, 1981), and this is supported by date from the M.R.C. Leukaemia Unit which found T-CLL in only 1.5% of 600 cases of CLL examined by marker studies (D. Catovsky, unpublished). Amongst the published reports of T-CLL a variety of clinical and morphological entities have been described including T prolymphocytic leukaemia (TPLL) (Brouet et al. 1975) and adult T cell disease in Japanese (Uchiyama et al, 1977) and West Indian Caribbean groups (ATLL) (Catovsky et al, 1982). In the original series of Brouet & Seligmann (1981) the group was defined as presenting in middle age with marked hepatosplenomegaly, some lymphadenopathy, skin involvement and with an aggressive disease course; peripheral blood and marrow lymphocytosis were variable.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Chronic T cell lymphocytosis: a review of 21 cases. 633 88


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>