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)

4 patients are described with rheumatoid arthritis, splenomegaly, neutropenia and an unusual proliferation of T cells in the blood and marrow. These patients are clinically similar to patients with classical Felty's syndrome but can be distinguished from them by staining blood and marrow mononuclear cells with a panel of monoclonal anti-T cell antibodies. The T cells from patients with T cell proliferations stain with UCHT1 (OKT3 equivalent) and UCHT4 (OKT8 equivalent but do not stain with a panel of OKT1-like antibodies. In 7 patients with classical Felty's syndrome there was no increase of UCHT4 cells in the blood and the large majority of T cells stained with OKT1-like antibodies. The marrows from the patients with T cell proliferations contain plentiful haemopoietic progenitor cells and it is probable that the T lymphocytes suppress their normal maturation. There was a poor response to splenectomy in 2 patients with T cell proliferations, and single cytotoxic drug therapy may be more appropriate when therapy is required. The literature is reviewed and it is suggested that the T cell proliferations may be secondary to the rheumatoid process.
 ...
PMID:Unusual T cell proliferations and neutropenia in rheumatoid arthritis: comparison with classical Felty's syndrome. 633 54

With the purpose of detecting granulocyte-specific membrane-directed autoantibodies, IgG was isolated from 14 patients with Felty's syndrome, from 5 patients with rheumatoid arthritis associated with neutropenia, and from 3 rheumatoid factor positive patients with autoimmune neutropenia. By means of indirect immunofluorescence suspensions of leukocytes from healthy controls were tested for their ability to bind whole IgG fractions or F(ab')2 fragments of IgG isolated from the neutropenic patients. By this method, whole IgG preparations from neutropenic patients and normal controls were invariably found to bind to the surface of granulocytes and a minor proportion of lymphocytes. In contrast. F(ab')2 fragments of IgG from the neutropenic patients failed to bind to the surface of granulocytes. Both whole IgG fractions and F(ab')2 fragments displayed displayed antinuclear antibody activity ruling out the possibility of loss of antibody reactivity during the preparation of F(ab')2 fragments. The neutropenia seen in rheumatoid arthritis with or without splenomegaly thus does not seem to be induced by granulocyte-specific membrane-directed IgG autoantibodies, but rather by a non-specific attachment of IgG most probably in immune complex bound form to Fc gamma receptors on neutrophils which thereby acquire surface properties facilitating removal from the circulating blood cell pool.
 ...
PMID:Lack of evidence for granulocyte specific membrane-directed autoantibodies in neutropenic cases of rheumatoid arthritis and in autoimmune neutropenia. 634 99

Since the approval of lithium use in treatment of acute mania, there have been numerous clinical trials of lithium in medical and psychiatric disorders. This paper gives a brief review of the literature on lithium trials in approximately fourteen medical conditions. These are: hyperthyroidism, metabolizing thyroid cancer, syndrome of inappropriate secretion of antidiuretic hormone, premenstrual tension syndrome, anorexia nervosa, Felty's syndrome, chemotherapy-induced neutropenia, aplastic anemia, seborrheic dermatitis, eczematoid dermatitis, cyclic vomiting, diabetes mellitus and asthma. Most of the case reports cited showed the efficacy of the side effects from lithium salt in the management of the symptoms and signs of these disorders, however, well-designed and controlled studies give negative results. The positive results are reported in the group of disorders having an underlying subdromal affective syndrome such as premenstrual tension syndrome and anorexia nervosa. Other encouraging reports include the effect of lithium to induce leucocytosis in Felty's syndrome and chemotherapy-induced neutropenia.
 ...
PMID:A review of clinical trials of lithium in medicine. 639 35

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
 ...
PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

A 64-year-old woman with rheumatoid arthritis developed Felty's syndrome. Her serum contained large amounts of IgA rheumatoid factor (RF) but insignificant levels of IgM-RF and IgG-RF. It is postulated that the high levels of IgA-RF may have contributed to the neutropenia.
 ...
PMID:Felty's syndrome associated with high levels of IgA rheumatoid factor. 674 12

A patient with Felty's syndrome complicated by severe infections, and characterized by marked hyperimmunoglobulinemia, circulating immune complexes, and high titers of rheumatoid factor is described. The clinical features, hematologic and immunologic abnormalities improved dramatically with steroids and were extended with gold therapy. Mechanisms of neutropenia and neutrophil dysfunction are discussed and a rationale for the treatment given.
 ...
PMID:Felty's syndrome: case report and rationale for disease-suppressant immunosuppressive therapy. 679 82

Twenty patients with Felty's syndrome were investigated. Isotopic studies of polymorphonuclear neutrophils, bone marrow biopsies and autoradiographies, and cultures of granulous stem cells showed that neutropenia resulted from three mechanisms acting simultaneously: hypermargination of the neutrophils predominantly in the spleen, decreased production of granulocytes in the bone marrow, and peripheral hyperdestruction of the neutrophils. Anti-granulocyte antibodies were detected in 3/12 patients. Other factors present in the serum of 2/4 patients seem capable of inhibiting the growth of granulocytic stem cells. Secondary bacterial infection (77%) may explain the severity of the prognosis: 13 out of 27 patients died 4 years on average after neutropenia was diagnosed.
 ...
PMID:[Mechanisms and prognosis of neutropenia in Felty's syndrome. 27 cases (author's transl)]. 709 32

A 63 year-old female patient with rheumatoid arthritis and positive rheumatoid serology presented persistent neutropenia without spleen enlargement detectable by either physical or isotopic means. A pattern identical to that described for classical Felty's syndrome was found on determination of the IgG bound to granulocytes by inhibition of the hemolysis of sheep erythrocytes previously incubated with human IgG. Inhibition of hemolysis in the patient was 94 %, 63 %, and 32 % (according to the concentration of granulocytes per mm3), while in the healthy control it was 37.5 %, 30 %, and 0 %, respectively. In spite of the absence of spleen enlargement, the patient was diagnosed as suffering from Felty's syndrome without splenomegaly. The diagnostic and follow-up value of the measurement of IgG bound to granulocytes is emphasized.
 ...
PMID:[Felty's syndrome without splenomegaly. A case report (author's transl)]. 720 85

The amount of IgG in the serum of patients with suspected immune neutropenia that binds to normal paraformaldehyde-fixed human granulocytes was measured simultaneously by a quantitative antiglobulin consumption assay and by binding of 125I-staphylococcal protein A (SPA). There was a significant linear relationship between the results of these two assays for the sera of 42 different patients. However, SPA binding appeared more sensitive than the quantitative antiglobulin assay for determining IgG antigranulocyte antibodies in serum. In a patient with Felty's syndrome who underwent splenectomy, the results of both assays on sequential serum samples correlated with clinical improvement. Thus, SPA binding appears to be a sensitive and reliable technique for measuring antigranulocyte antibodies, and there is a close correlation between antibody measured by antiglobulin consumption and those detected by SPA binding.
 ...
PMID:Human IgG antigranulocyte antibodies: comparison of detection by quantitative antiglobulin consumption and by binding of 125I staph protein A. 727 May 47

The objective of these studies was to assess the relative role of humoral or cellular immune mechanisms in the neutropenia of Feltry's syndrome. The amounts of neutrophil-bound IgG were measured by a sensitive antiglobulin inhibition assay. These values were increased in eight out of seventeen patients with Felty's syndrome over the highest values seen with seventeen patients with rheumatoid arthritis without leukopenia or twenty-six normal individuals. The levels of serum IgG neutrophil-binding activity from thirteen out of twenty-one patients with Felty's syndrome were greater than the highest values seen with normal sera compared to six out of thirty sera from patients with rheumatoid arthritis alone. A strong correlation was observed between the levels of direct neutrophil-bound IgG and the serum IgG neutrophil-binding activity for the seventeen patients with Felty's syndrome. Inhibition of normal marrow granulocyte colony growth greater than 40% was seen with the peripheral blood mononuclear leucocytes from five out of eleven patients with Feltry's syndrome. All five patients had normal levels of neutrophil bound IgG and normal to moderately increased levels of serum IgG neutrophil binding activity. In contrast, of the six patients whose mononuclear leucocytes did not inhibit granulocyte colony growth, three had markedly elevated levels of neutrophil-bound IgG and four had elevated levels of serum IgG neutrophil binding activity. These results suggest that humoral and cellular immune mechanisms of neutropenia may be present in different subsets of patients with Felty's syndrome.
 ...
PMID:Humoral and cellular immune mechanisms of neutropenia in patients with Felty's syndrome. 738 3


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