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

Some patients with chronically elevated large granular lymphocyte (LGL) numbers have rheumatoid arthritis (RA). Since these patients also may have neutropenia and splenomegaly, their symptoms resemble those of patients diagnosed as having Felty's syndrome (FS). We studied the immunophenotypic and genotypic characteristics of mononuclear cells from patients with RA and neutropenia to better determine the extent of heterogeneity in this condition. Four patients had markedly increased numbers of LGLs, which expressed HNK-1 antigen and IgG Fc receptors. In contrast, the remaining 8 patients, who had FS, had normal LGL counts, and surface marker studies showed normal numbers of HNK-1 and IgG Fc receptor positive cells. Clonal rearrangement of the T cell receptor beta chain gene was demonstrated in all 4 patients with excess LGLs, whereas a germline configuration of this gene was present in all 6 FS patients in whom this was studied. These results suggest that there are diverse groups among patients with RA and neutropenia. Since prognosis may differ, it is important to recognize that some patients who are considered to have Felty's syndrome may have a clonal proliferation of LGLs.
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PMID:Clonal proliferation of large granular lymphocytes in rheumatoid arthritis. 334 30

Sera of 22 patients with Felty's syndrome and 14 patients with rheumatoid arthritis were tested in assays routinely used for the detection of neutrophil antibodies (i.e. immunofluorescence, agglutination and cytotoxicity tests) as well as in the antibody-dependent lymphocyte-mediated granulocytotoxicity test. One or more of the routinely used assays were positive in a high percentage of the sera (77% and 64%, respectively). The antibody-dependent lymphocyte-mediated granulocytotoxicity test was only positive with sera of three patients with Felty's syndrome. Positive results in the immunofluorescence and agglutination tests could be attributed to the presence of immune complexes in the sera, whereas positive antibody-dependent lymphocyte-mediated granulocytotoxicity tests were probably due to the presence of HLA alloantibodies. It is concluded that serological tests routinely used for the detection of neutrophil autoantibodies should be interpreted with caution in patients with Felty's syndrome. Our results also indicate that the neutropenia in Felty's syndrome is rarely, if ever, due to neutrophil-specific autoantibodies.
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PMID:Lack of evidence for the presence of neutrophil autoantibodies in the serum of patients with Felty's syndrome. 334 94

The authors report 18 cases of Felty's syndrome followed, in an average, for 5 years (1 to 12 years). There were 3 deaths, 9 patients are in complete remission, 6 others still show signs of the disease: splenomegaly (4 cases), leucopenia (1 case) and only one complete Felty's syndrome. Steroid therapy has proved to be effective regardless of the mechanism of the neutropenia specified in 9 cases by an isotopic study. Prognosis and infectious risk are difficult to determine but the overall course was rather favorable.
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PMID:[Mechanism of Felty's syndrome and long-term course]. 337 72

The Tc-99m sulfur colloid liver-spleen scintigrams of nine patients with Felty's syndrome (a triad of rheumatoid arthritis, splenomegaly, and neutropenia) were reviewed. The characteristic scintigraphic findings include (1) moderate or severe splenomegaly, (2) the reversal of liver-to-spleen uptake ratio despite normal liver function tests, and (3) virtually no visualization of the bone marrow uptake and no pulmonary radiocolloid sequestration. In a late stage of cirrhosis of the liver, moderate-to-severe splenomegaly with the reversal of liver-to-spleen uptake ratio almost always accompanies abnormal liver function, and sometimes there may be associated pulmonary radiocolloid uptake. It was therefore concluded that in a proper clinical setting, a radiocolloid scintigraph may differentiate between Felty's syndrome and cirrhosis of the liver in a late stage.
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PMID:Radiocolloid scintigraphy in Felty's syndrome. 348 13

The authors have studied the case of a female patient with rheumatoid polyarthritis, who developed a lymphocytic proliferation in the blood, the marrow, and the liver, associated with a neutropenia. Several similar cases have been recently reported in the literature. The cellular proliferation is made of large granulous lymphocytes and the study of membrane markers enables to find the following homogeneous phenotype: E rosette+, CD8+, HNK-1+, FcR+, CD4-luminal diameter "divided by degrees - -, IgS-, HLA class II-. This lymphocytic sub-population produces little interleukin-2, responds weakly to mitogens (PHA, CON A, PWM), and inhibits the response of normal lymphocytes to the same mitogens. These lymphocytes have a weak natural killer activity but, on the contrary, develop a very strong cytotoxic activity which is antibody-dependent. Clinically, splenomegaly, anemia and infections are frequent and hepatomegaly or thrombopenia more rare. Adenopathies are never present. The evolution is chronic in nature and not very aggressive, although the lymphocytic proliferation is monoclonal in origin, as demonstrated in molecular biology studies. The neutropenia might be secondary to an inhibiting effect of lymphocytes on the granular precursors of the bone marrow. There is a definite association between this lympho-proliferative syndrome and rheumatoid polyarthritis, and this association appears to be different from the Felty's syndrome.
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PMID:[Characterization of chronic lymphocytic proliferation in a female patient having rheumatoid polyarthritis with neutropenia]. 361 55

LGL leukemia results from a chronic, clonal proliferation of LGL. Chronic neutropenia with recurrent bacterial infection and splenomegaly are common clinical manifestations. Rheumatoid arthritis coexists in some of these patients, who thus resemble patients with Felty syndrome. Other hematologic abnormalities that may occur include pure red-cell aplasia and adult-onset cyclic neutropenia. Lymphoid infiltration of bone marrow, splenic red pulp cords, and hepatic sinusoids is characteristic; lymph node and skin involvement are rare. Multiple serologic abnormalities are frequently present, including positive tests for rheumatoid factor and/or antinuclear antibody, polyclonal hypergammaglobulinemia, and circulating immune complexes. Antineutrophil and antiplatelet antibodies are often present. Leukemic LGL exhibit phenotypic heterogeneity; the most common phenotype in our patients is CD2+, CD3+, CD8+, HNK-1+, CD16-. Despite markedly increased numbers of LGL, functional activity of the cells is usually decreased. The mechanism of cytopenias is uncertain: in pure red-cell aplasia, it appears to be due to suppressive effect on erythropoiesis by abnormal LGL, but in patients with chronic neutropenia it may be antibody-mediated. Although most patients appear to have a relatively benign clinical course, mortality from infections and progressive lymphoproliferation is substantial. Optimal therapy remains undefined. Some preliminary evidence suggests that LGL leukemia may be associated with infection with a retrovirus similar to HTLV-I. Although relatively rare, LGL leukemia is of interest because a better understanding of this disease process may contribute to our knowledge of autoimmune diseases, the immunoregulatory functions of LGL, and the mechanisms controlling normal hematopoiesis.
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PMID:Large granular lymphocyte leukemia. Report of 38 cases and review of the literature. 362 48

The effect of the injection of serum from patients with rheumatoid arthritis (RA) and Felty's syndrome (FS) into mice on the number of circulating polymorphonuclear cells (PMN) was studied. The number of circulating PMN dropped to 61% (range 34-98%) of the initial counts after the injection of FS serum. This phenomenon was observed less frequently after injection of RA serum. In contrast, injection of serum from healthy controls always resulted in an immediate increase in the number of circulating PMN. No decrease in PMN counts was found after injection of FS sera pretreated with polyethylene glycol to precipitate immune complexes (IC). Gel filtration of FS sera on Sepharose 4B showed that the effect on the PMN counts in mice did not coincide with the 7S peak but occurred only in fractions containing larger material. Serum fractions from FS patients that contained IC were more active in producing neutropenia than the corresponding fractions from patients with RA. Microscopic and immunohistochemical examination of the organs from mice injected with FS serum showed sequestration of PMN and deposition of human IgG, IgA, and IgM in the vascular bed of the lungs. These results indicate that the interaction between PMN and IC of patients with FS leads to sequestration of PMN in mice and suggests that this interaction in humans may have a role in the pathogenesis of FS.
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PMID:Immune complexes and the pathogenesis of neutropenia in Felty's syndrome. 374 Sep 99

Recent studies of methotrexate in the treatment of patients with rheumatoid arthritis have precluded patients with neutropenia. We present a patient with rheumatoid arthritis complicated by severe neutropenia and recurrent infections, who was treated with low-dose methotrexate, orally, for 30 months. The patient experienced symptomatic improvement and a significant increase in granulocyte count, and the dosage of steroids was reduced. Low-dose oral methotrexate may be a therapeutic option in select patients with Felty's syndrome.
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PMID:Treatment of Felty's syndrome with low-dose oral methotrexate. 374 2

Immunofluorescence on polymorphonuclear cells (PMN) of patients with Felty syndrome (FS) revealed increased amounts of IgG, IgA, and IgM bound to the PMN surface compared with PMN of patients with rheumatoid arthritis alone. A positive correlation was found between the score for surface-bound immunoglobulins on FS-PMN and the results of the Clq binding assay in FS sera. After preincubation with sera from 20 patients with FS, immunofluorescence on PMN from healthy controls (HC) showed that these cells had bound IgG, IgA, and IgM. However F(ab')2 fragments of IgG from FS sera did not bind to PMN, although the antigen-binding reactivity of the F(ab')2 fragments was maintained as shown by control experiments. Immunoglobulins eluted from FS-PMN failed to bind to HC-PMN, whereas the corresponding IgG of patients with autoimmune neutropenia was bound. Gel filtration of FS sera on Sepharose 4B showed that the binding of IgG in FS sera to PMN did not coincide with the 7S peak but occurred mainly in fractions containing larger material. No binding of IgA and IgM to HC-PMN was found after incubation with FS sera pretreated with polyethylene glycol (PEG) to precipitate immune complexes. These results indicate that in sera of patients with FS the PMN-binding reactivity of IgG, IgA, and IgM is due to the binding of immune complexes containing these immunoglobulins and not to presence of autoantibodies directed to antigens on the neutrophil surface.
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PMID:Felty syndrome: autoimmune neutropenia or immune-complex-mediated disease? 388 Jan 80

The records of 25 patients who developed neutropenia (granulocyte count less than or equal to 2500 mm3) while receiving intramuscular gold sodium aurothiomalate (GSTM) were reviewed. According to commonly used clinical criteria, 3 patients developed Felty's syndrome, 8 gold myelotoxicity, and 14 mild, chronic benign granulocytopenia. Myelotoxicity occurred exclusively during the initial course of therapy (less than or equal to 1 g GSTM). Twelve of the patients with chronic granulocytopenia continue to receive gold without other signs of serious toxicity. We conclude that Felty's syndrome can develop during gold administration, and that many patients may continue safely to receive gold despite neutropenia.
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PMID:Neutropenia occurring during the course of chrysotherapy: a review of 25 cases. 393 31


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