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)

A-35-year-old woman with a long-lasting history of neutropenia and recurrent infections was found to have defective neutrophil chemotaxis, random motility, and in vivo migration. Although the bone marrow granulocyte reserve was normal, the patient failed to release an appropriate amount of granulocytes after injection of etiocholanolone. These features are characteristic of the so-called "Lazy leukocyte syndrome". The clinical presentation of the five cases of this syndrome so far reported and its pathophysiological aspects are discussed.
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PMID:Lazy leukocyte syndrome. 49 7

Since circulating and marginated human granulocytes are in rapid kinetic equilibrium, cells of these two compartments have been considered to be a homogeneous population. Our studies on the relationship between neutrophil maturity and the granulocyte alkaline phosphatase (GAP) activity cast doubt on this assumption. After iv administration of hydrocortisone, 12 male volunteers showed an augmentation in circulating granulocytes of 5730 cells/mm3, accompanied by an increase of band neutrophils from 18% to 33% (p less than 0.001). During this influx phase, the GAP activity decreased by 73% when measured cytochemically and by 28% when assayed biochemically (p less than 0.001 and less than 0.01, respectively). When granulocytes were demarginated by epinephrine, the mean count increased by 38%, accompanied by a rise in the portion of segmented forms from 74% to 79% (p less than 0.005) and by an increase of the cytochemical GAP activity by 24% (p less than 0.01). Exact complementary results were obtained during an excessive transient margination, the hemodialysis neutropenia: bands increased from 24% to 54% (p less than 0.02), while the cytochemical GAP dropped by 40% (p less than 0.005). Thus, our analysis of three situations with an acute transient shift of granulocytes indicates that functionally or chronologially "older" cells have a higher GAP activity, and that the transfer of granulocyte from the circulating to the marginal pool is selective.
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PMID:Disparity between circulating and marginated neutrophils: evidence from studies on the granulocyte alkaline phosphatase, a marker of cell maturity. 54 24

Granulocyte replacement therapy for the infected patient with severe neutropenia is effective in improving the short-term and possibly the long-term prognosis. The best methods of procuring and administering granulocytes still are under investigation, and no reliable method for predicting compatibility has been developed. At present granulocyte transfusion therapy is indicated for severely neutropenic patients with documented infection as an adjunct to antibiotic therapy. It should be used only at institutions that offer the necessary professional and technical skills to minimize danger and discomfort to both donor and recipient as well as the expense of the treatment.
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PMID:Granulocyte transfusion therapy: experimental basis and clinical applications. 58 13

Optimum serologic reactivity is observed if papain-treated granulocytes are reacted with cytotoxic antibody at low (5 degrees C) rather than warm (22 degrees C) precomplement incubation temperatures. Favorable in vitro conditions have allowed the identification of cytotoxic granulocyte antibodies in approximately 12% of nonimmunized normal males and females. Furthermore, the incidence of granulocytotoxic antisera in a group of alloimmunized patients did not exceed that observed in the normal population. In two cases cited, a normal male and a patient with pathologic neutropenia, cytotoxic antibodies against allogeneic granulocytes were autoreactive against the autologous cells of the serum producer. In the latter subject, an inverse association was demonstrated between the presence of autoantibody and the circulating neutrophil count. The incidence of granulocytotoxins in various diseases has been given and appears raised in systemic lupus erythematosus and asthma.
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PMID:Autoimmune cytotoxic granulocyte antibodies in health and disease. 60 49

Sera from two patients with granulocytopenia associated with collagen vascular disease caused the destruction of normal human granulocytes by autologous lymphocytes in vitro. Granulocyte cytotoxicity was measured by the release of 51Cr during incubation with test sera and lymphocytes in microtiter plates. Between 8% and 46% granulocytoxicity was produced in granulocytes from 8 normal donors by the sera from these two patients. Less than 6% granulocytotoxicity was seen with the sera from 14 normal subjects and 29 patient controls. Treatment of lymphocyte preparations with carbonyl iron and magnetic separation to remove phagocytic cells or treatment with complement-coated red cells followed by repeated gradient centrifugation to remove complement receptor-bearing lymphocytes did not reduce the granulocytotoxicity. There was a dose-response relationship between the concentration of positive sera and granulocytotoxicity. When these sera were fractionated by Sephadex G-200 gel filtration and by ion-exchange chromatography with DEAE-cellulose, the active component appeared in the IgG-containing fractions. Thus, IgG antibody-dependent, lymphocyte-mediated granulocyte cytotoxicity represents a means of detecting human granulocyte antibodies and is a possible mechanism of autoimmune neutropenia in these two patients.
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PMID:Antibody-dependent lymphocyte-mediated granulocyte cytotoxicity in man. 61 59

Myelopoiesis was assessed in 41 untreated patients with widespread cancer without evidence of infection. None of these patients had neutropenia, although leukopenia due to decreased concentrations of blood lymphocytes was observed in two. Sixteen patients (39%) had neutrophilia and 12 patients (29%), 5 of them with neutrophilia, had decreased marrow granulocyte reserve. Neither the blood neutrophil counts nor the marrow granulocyte reserve correlated significantly with the marrow myeloid mitotic indices, myeloid to erythroid ratio or the number of marrow colony-forming cells. There was no difference in the duration of the disease, the extent of systemic metastasis and the degree of malnutrition between patients with or without myelopoietic abnormalities. Our findings suggest that decreased marrow granulocyte reserve and neutrophilia are common in untreated patients with disseminated carcinoma.
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PMID:Myelopoietic abnormalities in patients with metastatic carcinoma. 63 62

An adult with long-standing neutropenia had the functional granulocyte abnormalities typical of the lazy leucocyte syndrome. Scanning electron microscopy of the patient's neutrophils showed alteration in the surface configuration of the cell with coarsening of the normal fine ruffles and the appearance of knob-like projections. Similar functional and anatomical changes were induced in normal neutrophils by treatment with vinblastine. The lazy leucocyte syndrome may be a consequence of altered membrane microfilamentous protein structure or function, and undue rigidity of the affected neutrophils may explain the clinicopathological features of the disease.
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PMID:Lazy leucocyte syndrome--disorder of the granulocyte membrane? 64 Dec 7

Studies were done of cell production by marrow in diffusion chambers implanted in the peritoneal cavity of rabbits subjected to various stimuli to hematopoiesis. In chambers in neutropenic hosts and in hosts injected with endotoxin, animals presumed to have an increased stimulus to granulopoiesis, there was increased production of granulocytes but there was also increased production of red cells. Although red cell production was decreased in chambers in polycythemic hosts, granulocyte production was not different from that in controls. Stimulation of erythropoiesis by erythropoietin injections or by exposure to hypoxia increased red cell production by marrow in the implanted diffusion chambers without diminishing granulopoiesis. Only in chambers in hosts made anemic by bleeding was there an increase in red cell production accompanied by a decrease in granulocyte production. In these anemic hosts induction of neutropenia led to an increase in granulopoiesis without any depression of erythropoiesis.
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PMID:Marrow culture in diffusion chambers in rabbits. II. Effect of competing demands for red cell and white cell production on cell growth. 64 17

A case of neutropenia in an infant is described, associated with repeated minor infections which responded to treatment with antibiotics. A granulocyte agglutinin was detected in the serum. Following treatment with prednisolone the neutropenia remitted, the antibody disappeared and the infections ceased. Treatment was discontinued after three months and the child remained well five months later. Reports of similar cases are reviewed and the significance of the antibody discussed.
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PMID:Auto-immune neutropenia in an infant. 66 93

The mechanism of granulocyte depletion in a patient with systemic lupus erythematosus and neutropenia was investigated. Neutrophil kinetic studies showed a shortened intravascular survival (t1/2 of 1.6 hours) in the face of an increased marrow neutrophil pool. IgG bound to the patient's neutrophils, measured by the Fab antiF(ab')2 assay, was nearly three times normal. The IgG neutrophil-binding activity of the patient's serum was elevated in serial samples obtained over two years. In addition, his serum was able to opsonize normal neutrophils for ingestion by other neutrophils as detected by 14C-1-glucose oxidation. Enhanced IgG PMN-binding activity was observed with sucrose density gradient fractions of the patient's serum containing either large complexes (19S or greater in size), intermediate complexes (between 7S and 19S), or monomeric IgG. Only the momomeric IgG fraction from the patient's serum, however, opsonized normal neutrophils for ingestion by other neutrophils. These results support the hypothesis that anti-cell antibodies were responsible for the neutropenia in this patient by opsonizing neutrophils for ingestion by other phagocytic cells.
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PMID:Autoimmune neutropenia in systemic lupus erythematosus. 66 71


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