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

Thirty-two patients with the Felty syndrome, defined by the presence of rheumatoid arthritis, splenomegaly, and neutropenia, have been studied in comparison with 32 patients with rheumatoid arthritis matched for age, sex, and disease duration, and 9 patients with rheumatoid arthritis and idiopathic neutropenia. Patients with the Felty syndrome had severe destructive arthritis, which progressed during follow-up despite little evidence of objective synovitis, and a higher frequency of extra-articular manifestations, including vasculitis. Bacterial infection tended to occur in patients with the lowest neutrophil count but continued to occur in some despite normalization of the WBC. Prognosis was poor and 8 deaths occurred, predominantly from sepsis. Serologic features were prominent. High titers of IgG rheumatoid factor and circulating immune complexes characterized patients with persistent neutropenia. A family history of rheumatoid arthritis was more common in patients with the Felty syndrome. The association with HLA DR4 was very strong; in addition there was an increased frequency of the DQw3 variant, 3b, suggesting that HLA Class II genes in linkage with DR4 may contribute to disease expression.
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PMID:The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. 196 4

A significant association of autoimmune neutropenia (AIN) in infants due to NA1-specific autoantibodies and HLA-DR2 is reported. Nineteen of 26 infants presenting with AIN and NA1 autoantibodies possessed the DR2 antigen, while only one out of six children with AIN, but non-NA1-specific autoantibodies, was DR2 positive. Furthermore, one adult patient with primary biliary cirrhosis and periods of neutropenia due to NA1 autoantibodies also carried the DR2 antigen. All DR2-negative patients were positive for DRw6. These findings indicate that a close relationship exists between autoimmunization against the NA1 antigen and HLA-DR2 and possibly also DRw6.
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PMID:Autoimmunization against the neutrophil-specific NA1 antigen is associated with HLA-DR2. 200 73

A 17-year-old boy developed autoimmune pancytopenia in the absence of chronic graft-versus-host disease 170 d after allogeneic bone marrow transplantation (BMT) from his HLA identical brother. The anaemia and thrombocytopenia responded to conventional immunosuppressive treatment, but the neutropenia was refractory to this and to splenectomy and subsequent removal of splenic remnant. Following total lymphoid irradiation the neutrophil count rose to low normal levels but thrombocytopenia and anaemia secondary to marrow hypoplasia required transfusion support. Bone marrow function was finally normalized by an additional transfusion of donor marrow without prior immunosuppressive therapy. We conclude that late onset immune pancytopenia post BMT caused by antibodies of probable donor origin may be life threatening in the absence of chronic graft-versus-host disease.
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PMID:Late onset immune pancytopenia following bone marrow transplantation. 153 5

A total of 304 children under the age of 15 years with acquired severe aplastic anemia (SAA) received immunosuppressive therapy (IS) (n = 133) or a matched bone marrow transplant (BMT) (n = 171). The projected 10-year survival is 48% and 63% respectively (p = 0.002). Results following BMT have improved considerably over the years from 49% in 1970-80, to 70% in 1981-83 (p = 0.002) and to 81% between 1984-88 (p = 0.08). Other favorable prognostic factors are the use of cyclosporin A (p = 0.004), no previous therapy (p = 0.006) and early BMT (p = 0.009). In multivariate analysis only the year of treatment proved significant (p = 0.02). In contrast, results of IS are greatly dependent on the severity of pre-treatment neutropenia with survival of 56% versus 37% for neutrophils more or less than 0.2 x 10(9)/l (p = 0.003). Poor survival was associated in univariate analysis with female sex (43%), post-hepatitis SAA (37%), children not receiving androgens (38%) and patients younger than 5 years (35%), especially if associated with a low neutrophil count (11%). In multivariate analysis only the degree of neutropenia proved significant (p = 0.005). These results suggest that IS is a satisfactory alternative therapy for children with moderately SAA in the absence of an HLA-identical sibling, although BMT remains the treatment of choice. In children under 5 years with very SAA, results with IS are so poor that a search for an unrelated matched donor is justified as early as possible.
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PMID:Treatment with marrow transplantation or immunosuppression of childhood acquired severe aplastic anemia: a report from the EBMT SAA Working Party. 225 62

Thirteen HLA-identical bone marrow donors served as the sole source of daily granulocyte transfusions for respective marrow recipients during the period of severe neutropenia between transplantation and engraftment. They experienced 12 to 29 (median, 17) daily, continuous flow centrifugation leukapheresis procedures using hydroxyethyl starch, but no corticosteroids, with little serious difficulty. No immediate clinical reactions occurred in 90 percent of 228 procedures. Mild citrate reactions were noted in 9 percent, and only two procedures (0.8%) were discontinued due to severe reactions. Ten donors (77%) answered a questionnaire mailed weeks later, and six reported transient, late clinical adverse effects. Five had moderate dermatologic problems; one had minimal hypertension requiring no therapy. Donors were monitored daily for laboratory abnormalities while donating granulocytes. Hemoglobin concentration and platelet counts remained stable (autologous red cell transfusions had been given). Blood leukocyte counts gradually fell (p less than 0.05), particularly after 10 or more daily granulocyte donations, and this fall was associated with a decrease of about 33 percent in leukocyte yields. No attempts were made to improve yields by giving higher doses of hydroxyethyl starch or by corticosteroid stimulation. With primary emphasis on donor safety, it seems feasible for a few compatible donors to provide prolonged granulocyte transfusion support for designated patients. However, diminishing leukocyte yields may result from intensive, repeated leukapheresis.
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PMID:Effects of intensive granulocyte donation on donors and yields. 242 11

A 37-year-old man with severe transfusion-requiring anemia and neutropenia associated with lymphocytosis of large granular lymphocytes (LGLs) has been followed over a period of 3.5 years. Detailed phenotyping of the LGL has been performed, revealing a phenotype of CD3+, CD8+, CD16+, HLA-DR+, and Leu-7+. Southern-blot analysis of the T-cell receptor beta-chain locus detected a gene rearrangement, thus providing proof of monoclonality of the peripheral blood LGLs.
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PMID:Lymphocytosis of large granular lymphocytes associated with anemia and neutropenia: proof of monoclonality of the LGL-population, but benign clinical course. 254 68

A boy with combined immunodeficiency having low natural killer (NK)-cell activity received thymopoietin pentapeptide (TP-5) treatment, transplanted with T cell-depleted HLA-haploidentical bone marrow (BMT) cells from his father and with thymus tissue from an infant at different times during the first year of life. He showed a marked increase in large granular lymphocytes (LGL) both during the treatment with TP-5 and after BMT. The LGL generated following TP-5 injection had a T3+Leu11- surface phenotype and low NK activity. In contrast, the LGL appearing after BMT showed T3-, Leu7+, and/or Leu11+ surface phenotypes, had high NK- and K-cell activities, and were lymphokine-activated killer (LAK)-cell precursors. These killer activities were assigned to the Leu7-Leu11+ subset and proved to be of recipient origin. LGL proliferation following BMT was accompanied by neutropenia, which was improved in association with a reduction in the number of LGL and the appearance of T cells of BMT donor origin following thymus transplantation. This suggested the inhibition of granulopoiesis by the LGL and an in vitro study revealed that the Leu7+Leu11- subset of LGL suppressed the growth of granulocyte/macrophage colony-forming units. These results indicated that phenotypically different LGL could be generated by different treatments and that the LGL showing NK activity were distinct from those regulating granulopoiesis. It was also suggested that the generation of LGL was controlled by T cells.
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PMID:Phenotypical and functional heterogeneity of the large granular lymphocytes increased after various treatments in a patient with combined immunodeficiency. 264 8

In order to obtain an estimate of the frequency of platelet-specific and granulocyte-specific antibodies and of the effect of such antibodies on the platelet count and granulocyte count of the newborn infant, serum from 147 women in their second or subsequent pregnancies was tested. No platelet-specific antibodies were found but 29 of the women had granulocyte-specific antibodies and the corresponding infants had granulocyte counts which were significantly lower than those of infants without antibodies. HLA antibodies were found in the sera of 57 women but were not associated with diminished platelet or granulocyte counts in the corresponding infants. Maternal granulocyte antibodies may be an underestimated contributory factor in the pathogenesis of neonatal neutropenia.
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PMID:Maternal alloimmunization to HLA, platelet and granulocyte-specific antigens during pregnancy: its influence on cord blood granulocyte and platelet counts. 291 19

Morphologically distinct lymphoid cells with homogeneous, condensed chromatin and scant cytoplasm can be observed in large numbers in the bone marrow of children with a variety of hematologic and nonhematologic disorders. In some patients, these cells may account for greater than 50% of the bone marrow cells, creating a picture that can be confused with acute lymphoblastic leukemia (ALL) or metastatic tumor. Although originally called hematogones (HGs), a variety of other names have been proposed for these unique cells. The clinical significance of expanded HGs has not been resolved, and the biologic features of these cells are incompletely described. In this study, we correlate the clinical, morphologic, cytochemical, flow cytometric, molecular, and cytogenetic properties of bone marrow samples from 12 children with substantial numbers of HGs (range 8% to 55% of bone marrow cells). Diagnoses in these patients included anemia, four; neutropenia, one; anemia and neutropenia, one; idiopathic thrombocytopenic purpura, two; retinoblastoma, two; Ewing's sarcoma, one; and germ cell tumor, one. Flow cytometric analyses of bone marrow cells demonstrated a spectrum extending from early B-cell precursors (CD10+, CD19+, TdT+, HLA-Dr+) to mature surface immunoglobulin-bearing B cells in these patients, corroborating our morphologic impression of HGs, intermediate forms, and mature lymphocytes. DNA content was normal, and no clonal abnormality was identified by either cytogenetic or immunoglobulin and T-cell receptor (TCR) gene rearrangement studies. Follow-up ranged from 3 months to 3 years. None of the patients has developed acute leukemia or bone marrow involvement by solid tumor. The possible role of HGs in immune recovery and hematopoiesis is presented.
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PMID:Hematogones: a multiparameter analysis of bone marrow precursor cells. 291 89

Sera were analyzed from patients who were suspected to have antibodies to neutrophils. The analysis comprised five methods which avoid heterologous antibodies to human immunoglobulin. These methods were the granulocyte agglutination test (GAT), the granulocyte cytotoxicity test (GCT), the monocyte cytotoxicity test (MCT), the lymphocyte cytotoxicity test (LCT) and immune phagocytosis inhibition test (IPI). Each serum was tested with cells from five healthy donors, at least, and some with cells from relatives. After exclusion of sera containing multiple antibodies and HLA-antibodies with positive LCT- and IPI-tests, the GAT- or GCT-reactive antibodies were significantly (p = 0.00005) more frequent among patients with neutrophil counts less than or equal to 1.0 X 10(9)/l (30%; n = 117) than among patients with neutrophil counts greater than 1.0 X 10(9)/l (9%; n = 111). Within the group of neutropenic patients (less than or equal to 1.0 X 10(9)/l) these antibodies were significantly (p = 0.001) more frequent in patients without (48%; n = 46) than with reduction of the granulopoiesis (13%; n = 31). This typical feature of an immune cytopenia also could be shown with GAT-reactive antibodies alone. From all five antibody tests the diagnostic criteria of the autoimmune neutropenia (AINP) and neonatal alloimmune neutropenia (NIN) were infered. 25 patients with clearly defined AINP presented with significantly more infectious complication than 23 patients with only assumed AINP (48% versus 13%). Further, the clinical findings of eight patients with NIN were described. --Antibodies only reactive in the GAT were detected in healthy individuals (1.3%; n = 75) and patients without indication for AINP or NIN (8%; n = 111), also.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Antibodies, clinical and hematologic findings in immunoneutropenias]. 291 53


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