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 retrospective study of 61 episodes of agranulocytosis observed in 56 patients between 1958 and 1977 is reported. The diagnosis was based on peripheral granulocyte counts below 500/mm3 and further documented by bone marrow analysis. The aim of this study was to determine whether the new guidelines for supportive care introduced in January 1973 led to an improvement in overall prognosis in this disease. Therefore, the patients were divided into two groups: the first group included 39 episodes of agranulocytosis observed between 1958-1972 and the second group 22 episodes observed between 1973-1977. Standard supportive care administered in the latter group included reverse isolation (hand-disinfection and gown-change before patient contact, conventional hospital single room), the immediate initiation of an appropriate combination of antibiotics in infectious states and additional granulocyte transfusions in selected cases. The two groups compared were similar as to the extent of neutropenia and the frequency of severe infectious complications. On the other hand, patients of the first group showed more advanced recovery of myelopoiesis as compared to the second group at the time of hospital admission. Death due to infection was observed in 36% of episodes in the first group, but only in 9% in the second group. The supportive care introduced in 1973 thus appears to improve the prognosis of agranulocytosis to a substantial extent.
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PMID:[Drug induced agranulocytosis. Improved prognosis due to better supportive care]. 67 12

In human bone marrow culture studies the number of granulocyte progenitor cells (CFUc) is expressed as the number of colonies per 10(5) incubated, nucleated cells. However, this provides little information on cell proliferation kinetics of granulopoiesis. The ratio of progenitors (CFUc) to end cells (metamyelocytes or granulocytes) is related to the effectiveness of granulopoiesis and this allows an estimate to be made of an amplification factor within the maturing and proliferating compartments. We measured an index equal to the number of CFUc per 10(5) falls when a large number of granulocytes is produced by each progenitor cell. This was observed in many patients with a neutropenia. An estimation of the total number of metamyelocytes is based on the ratio of the number of metamyelocytes to the number of blood granulocytes. This allows an estimate of the total number of CFUcs, which is of clinical interest.
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PMID:Ways of expressing results of human bone marrow progenitor cell culture. 70 35

The patterns and types of infection in 93 infectious episodes in 76 patients who received supportive granulocyte transfusions are presented. In this population of infected patients 86 per cent had debilitating malignancies, 88 per cent of the infectious episodes were associated with severe (less than 100/microleters) neutropenia and septicemia was documented in 56 per cent. The overall four-week survival was 71 per cent. Patients with localized infection did extremely well. Pediatric patients also responded well to the transfusion dose and schedule. Older patients (greater than 60) and patients over the age of 17 with diffuse infection did not do as well. Delay in the initiation of granulocyte transfusions after a diagnosis of serious infection was a significant factor in the group which died less than four weeks after the initial WBC transfusion. Donor reactions in nylon filtration leukapheresis and problems associated with administration of nylon filter cells are presented and discussed.
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PMID:Supportive granulocyte transfusion in the infected severely neutropenic patient. 72 15

The marginal granulocyte pool (MGP) was measured by epinephrine infusion in normal and neutropenic subjects. Neutrophil response curves to doses of 0.025 to 0.3 mg. in three normal subjects indicated that maximal neutrophil response was achieved by 0.1 mg. In 21 normal subjects, absolute neutrophils increased from 700 to 3,100 per microliter. The percentage increase ranged from 18 to 107 per cent of baseline. The per cent increase tended to be greater with low-normal baseline neutrophils than with high-normal neutrophils, although this relationship was not observed when increase was determined in absolute values. In neutropenic patients mean per cent increase of neutrophils was greater than observed in normal subjects, 121 vs. 50 per cent. Although the increase expressed in absolute neutrophil numbers was less in subjects with lower baseline neutrophil concentrations, there was an inverse correlation between the baseline neutrophil concentrations, there was an inverse correlation between the baseline neutrophils and the per cent increment following epinephrine. Mean increase was 200 per cent in patients with less than 200 neutrophils per microliter, compared with 61 per cent in patients with 1,000 to 1,500 neutrophils per microliter. These results indicate that circulating granulocyte pool (CGP) size may be misleading with respect to total blood neutrophils and in a sense confirm the concept of shift neutropenia, a decreased CGP and MGP as neutropenia becomes more profound suggests that shift neutropenia may be a normal physiologic methanism rather than a distinct neutropenic syndrome.
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PMID:Marginal neutrophil pool size in normal subjects and neutropenic patients as measured by epinephrine infusion. 78 57

During the course of granulocyte collection by continuous-flow filtration leukopheresis, an abrupt fall in neutrophil count was noted (mean decrease 77%, range 64%-95%). Neutropenia occurred within 5 min of return of blood exposed to the nylon fiber filters and lasted less than 30 min. Saline exposed to the fibers, withdrawal and reinfusion of whole blood, and heparin did not cause neutropenia. Heparinized blood passed by gravity through isolated filters and reinfused immediately also induced neutropenia (mean decrease 64% +/- 8%, range 11%-19%). Blood anticoagulated with ACD (decrease 19.5% +/- 6%, range 6%-56%), heparinized plasma (N = 10, decrease 15% +/- 3%, range 3%-29%) and platelet-rich plasma exposed to the filters failed to produce neutropenia. 91% +/- 2% of the neutrophils adhered to the fibers using heparinized blood as compared to 21% +/- 5% using ACD (p less than 0.001). All donors were asymptomatic during the infusions. These results suggest that during neutrophil adherence a substance is released which produces profound, transient neutropenia perhaps by inducing margination of cells.
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PMID:Transient neutropenia induced by transfusion of blood exposed to nylon fiber filters. 80 6

Twenty-three children with various stages and morphologic types of leukemia were treated with multiple granulocyte transfusions obtained by filtration leukapheresis when neutropenia-associated infection appeared unresponsive to antibiotics. All children meeting the above qualifications were given granulocyte transfusions during this time period. Twenty-one of 23 became afebrile during or shortly after the transfusions; one died with disseminated Herpes simplex; and one became well enough to be discharged, although he was never free of fever. Frequent mild to moderate fever and chills were noted. One child developed a severe pulmonary reaction followed by resolution of pneumonia. Filtration leukapheresis is a useful adjunct in controlling severe infections in neutropenic children.
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PMID:Granulocyte transfusions in children using filter-collected cells. 82 3

Two patients with kala-azar were studied, one with DF32P (diisopropylfluorophosphate) and one with 51CR (chromate), in an attempt to elucidate the mechanisms producing neutropenia in this disease. The granulocyte half-life was found to be reduced in both patients, with pooling and probable destruction occurring in the spleen and, to a lesser extent, in the liver. Bone marrow neutrophil reserve, estimated by the response to intravenous hydrocortisone hemisuccinate, was found to be markedly reduced in both patients. An enlarged marginal granulocyte pool in one patient indicated that the neutropenia may also be due to altered intravascular granulocyte distribution.
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PMID:Studies of the neutropenia in kala-azar: results in two patients. 84 53

Within six months of the introduction of the new antipsychotic drug clozapine in Finalnd, 17 cases of neutropenia or agranulocytosis were recorded amongst about 3000 patients treated. Agranulocytosis was fatal in eight patients, and in addition, two patients developed thrombocytopenia, and one patient leukaemia. As additional cases might well have been overlooked as banal infections, the risk of developing agranulocytosis during clozapine treatment was at least 0.5%. Impaired elimination of the drug or increased susceptibility of granulocyte precursors to clozapine, possibly due to some inherited characteristic, might explain the high incidence of complications.
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PMID:Agranulocytosis during treatment with chlozapine. 85 3

The congenital neutropenias are a heterogenous group of diseases whose etiology and pathogenesis are largely unknown. We studied nine neutropenic patients from seven families. Evaluation included peripheral blood cell and differential cell counts, epinephrine and typhoid vaccine stimulation studies. Rebuck skin windows, and bone marrow aspirations for morphological assessment and for in vitro culture in liquid suspension and in agar plates. Parallel cultures were set up with and without colony-stimulating activity (CSA), and peripheral leukocytes were assayed for cellular production of CSA. Patients were initially classified on the basis of their clinical course: benign, mild, moderately severe, or severe disease. One patient in the moderately severe group had an immunoglobulin disorder. Morphologically normal mature granulocytes were seen in bone marrow aspirates of two patients, and maturational defects of varying degree were seen in the remaining seven. Colony formation in agar was markedly reduced below normal in three of seven, moderately reduced in two of seven, and greater than normal in two patients. Colonies in six of seven patients consisted exclusively of macrophages. Marrow from all but one of the nine patients demonstrated poor neutrophil development in suspension culture, and addition of CSA did not result in augmented granulocytic proliferation or maturation. A scheme of normal neutrophil maturation is proposed, and the nine patients were categorized according to this scheme. Four patterns of congenital neutropenia emerged: type 1 was the most benign form of disease with essentially normal clinical and in vitro parameters, and a defect considered to be due to a small committed stem cell pool, abnormal release, or excessive utilization peripherally; type 2 had mild disease with presumed defective committed stem cell differentiation along the granulocyte line; type 3 included benign to severe clinical expression with an apparent defect at the level of the committed granulocyte precursor more severe than in type 2; type 4 disease had varied clinical expression but evidence for a defect at the level of the pluripotent stem cell.
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PMID:Evaluation of congenital neutropenic disorders by in vitro bone marrow culture. 85 38

A 10-year-old girl was first seen at age 5 years with pneumonia and neutropenia. Since then, she has remained leukopenic, although manifesting a leukocytosis only when she has pulmonary infection. A rapid fall in her peripheral WBC count occurs with initiation of antibiotic therapy. Despite her neutropenia, marked myeloid hyperplasia is evident on marrow smear examination; many cells being hypersegmented with fine intralobular bridging with chromatin strands and cytoplasmic vacuolation. The peripheral WBC response to epinephrine adminstration did not indicate a shift from the circulating to marginal neutrophil pool. Results from a Rebuck skin window test suggested poor neutrophil tissue migration. A defect in granulocyte release from the patient's marrow may explain these bizarre hematologic findings.
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PMID:'Myelokathexis'. Neutropenia with marrow hyperplasia. 86 17


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