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

Two cases of immune haemolytic anaemia in newborn infants who had received exchange transfusion on the first day of life because of hyperbilirubinaemia probably due to a conjugation defect, are reported. The first baby, born in the 30th week of gestation presented erythrocyte-fixed IgM antibodies + C, neutropenia, and circulating leucocytotoxic antibodies. The second baby, born at term to a diabetic mother showed erythrocyte-fixed IgG + IgM antibodies + C and a selective IgA deficit. In both cases the anaemia improved and the Coombs test became negative spontaneously in the 4th and 5th months of life, respectively. These haematologic alterations are similar to a graft versus host (GVH) reaction due to the persistence in the neonate blood of the donor's immunocompetent cells.
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PMID:Immune haemolytic anaemia after exchange transfusion. 30 19

Sera from 21 rheumatoid arthritis patients with accompanying neutropenia (less than or equal to 2000 neutrophils/microliter) and 45 rheumatoid arthritis patients without neutropenia were studied for the occurrence of IgD granulocyte-specific antinuclear antibodies. Such antibodies were found in 67 per cent of the neutropenic and 18 per cent of the non-neutropenic cases (p less than 0.001). The titres of IgD granulocyte-specific antinuclear antibodies varied independently of the titres of IgD and complement-fixing granulocyte-specific antinuclear antibodies, but showed some covariation with granulocyte-specific antinuclear antibodies of the IgA and IgM classes most probably reflecting a broad polyclonal antibody response to phagocyte nuclear antigens in the serologically highly active neutropenic cases. Gel filtration studies on selected sera containing IgD granulocyte-specific antinuclear antibodies indicated participation of these antibodies in immune complexes. It is thus possible that IgD granulocyte-specific antinuclear antibodies may have some significance for the rheumatoid inflammatory processes.
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PMID:The prevalence and possible significance of IgD granulocyte-specific antinuclear antibodies in neutropenic and non-neutropenic cases of rheumatoid arthritis. 30 70

Immunologic deficiency was suspected in an 18-month-old Standardbred horse with persistent fever, multifocal bacterial infection, and neutropenia with a large number of immature neutrophils. Serum protein electrophoresis revealed marked depression of the gamma-globulin fraction (0.2 g/100 ml). Immunologic testing and histologic examination of lymphoid tissues identified the immune deficit as agammaglobulinemia. Serum concentrations of immunoglobulin (Ig)G and IgG(T) were initially low and declined with time; IgM and IgA were not detectable. The horse failed to produce antibodies when inoculated with foreign antigens but had a positive cell-mediated skin reaction to intradermal phytolectin injection, and lymphocytes responded normally to in vitro stimulation by mitogens. Histologic examination of lymphoid tissues revealed absence of germinal centers and plasma cells.
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PMID:Agammaglobulinemia in a horse. 50 Apr 81

Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.
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PMID:Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis. 124 58

A case of chronic benign neutropenia is described in association with circulating leucoagglutinins. IgG and IgM leucoagglutinins demonstrated by Sephadex G-200 chromatography agglutinated neutrophils and monocyte enriched leucocyte preparations. Total IgG, IgA, IgM and IgE, total haemolytic complement, C3 and C4 complement components were all within normal limits. Lymphocyte populations and transformation were normal apart from a relative increase in the number of B lymphocytes. In vitro studies showed that the patient's monocytes responded in chemotaxis, phagocytosis and intracellular killing of S. aureus in a comparable fashion to that of control neutrophils. Although the patient's serum also agglutinated monocytes these findings do not exclude the possibility that leucoagglutinins may have aetiological significance in this disease. The study also emphasizes the versatility of the monocyte in benign neutropenia.
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PMID:Leucocyte function in a case of chronic benign neutropenia of infancy associated with circulating leucoagglutinins. 125 76

A 20-month-old Indian boy presented with recurrent pyogenic infections and failure to thrive. His IgG and IgA levels were low, but his IgM was elevated. He also had undetectable isohaemagglutinin titre and neutropenia, both parameters being poor prognostic indicators in this very rare primary immunodeficiency state--antibody deficiency with hyper IgM. Our patient subsequently succumbed to Pseudomonas aeruginosa septicaemia and meningitis inspite of aggressive antibiotic and intravenous gammaglobulin therapy. To the best of our knowledge, this is the first such case to be documented in Malaysia.
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PMID:Antibody deficiency with hyper IgM--a case report. 130 25

We carried out a study in patients with severe neutropenia from hematologic malignancy and suspected gram-negative sepsis to evaluate the clinical significance of endotoxin concentrations in plasma before and during a therapeutic intervention with a human polyclonal immunoglobulin M (IgM)-enriched immunoglobulin preparation (Pentaglobin; Biotest, Dreieich, Germany). Twenty-one patients with acute leukemia or non-Hodgkin's lymphoma entered the study upon the development of clinical signs of gram-negative sepsis and received the IgM-enriched immunoglobulin preparation every 6 h for 3 days (total dose, 1.3 liter with 7.8 g of IgM, 7.8 g of IgA, and 49.4 g of IgG), in addition to standardized antibiotic treatment. Concentrations of endotoxin and IgM and IgG antibodies against lipid A and Re lipopolysaccharide (LPS) in plasma were determined by a modified chromogenic Limulus amebocyte lysate test and semiquantitative enzyme linked immunosorbent assay, respectively, before each immunoglobulin infusion and during the following 25 days. Seventeen patients were endotoxin positive; in five of these patients, gram-negative infection was confirmed by microbiologic findings. Prior to therapy, endotoxemia correlated significantly with the occurrence of fever, and a quantitative correlation between the endotoxin concentration and body temperature was found during the individual course of infection in 8 of the 17 patients. Overall mortality from endotoxin-positive sepsis was 41% (7 of 17) and 64% (7 of 11) in patients with symptoms of septic shock. Nonsurvivors had significantly higher maximum concentration of endotoxin in plasma compared with those of survivors at the first study day (median of 126 versus 34 pg/ml; P < 0.05) and during the whole septic episode (median of 126 versus 61 pg/ml; P < 0.05). In survivors, immunoglobulin therapy resulted in a significant decrease in endotoxin levels in plasma within the initial 18-h treatment period, from a pretreatment median value of 28 pg/ml to a value of 8 pg/ml (P< 0.05). In the seven patients who died from uncontrollable infection, no effect of therapy on endotoxin levels in plasma was observed. IgM and IgG antibodies against lipid A and Re LPS increased significantly under immunoglobulin treatment, with significant correlations between antibodies against lipid A and Re LPS. These data strongly suggest a prognostic significance of the endotoxin levels in plasma and a potential effect of treatment with a polyclonal IgM-enriched immunoglobulin preparation. Further studies are needed to substantiate these findings and to assess the impact on the clinical course by way of a prospective placebo-controlled clinical trial.
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PMID:Endotoxin concentration in neutropenic patients with suspected gram-negative sepsis: correlation with clinical outcome and determination of anti-endotoxin core antibodies during therapy with polyclonal immunoglobulin M-enriched immunoglobulins. 144 93

The role of local immunity in relation to the frequent and heterogeneous pulmonary manifestations of HIV-1 infection in children is poorly understood. In order to examine lung immunity in pediatric AIDS patients, the cellular composition, immunoglobulin, and immune complex (IC) levels were evaluated in 23 samples of bronchoalveolar lavage (BAL) fluid and peripheral blood from 19 pediatric AIDS patients with acute pulmonary pathology. The patients were of two age groups: 4.0-21.5 months (N = 9) and 2.3-13.1 years (N = 10). In BAL, lymphocytes were elevated in 25-45% of samples, and neutrophils were elevated in 27-33%; BAL macrophages varied in percentage (28-99%) but had normal morphology. The blood differentials of pediatric AIDS patients undergoing BAL did not show significant differences when compared with a group of pediatric patients with tuberculosis, but leuko- and neutropenia was noted when compared with pediatric patients with pneumonia and no HIV disease. Of the immunoglobulins measured (IgG, IgM, IgA) only IgG was detectable in unconcentrated BAL fluid (1-37 mg/dl, equivalent to 12-630 mg/dl in the epithelial lining fluid after correction using urea as a marker of dilution). All patients were hypergammaglobulinemic and 83% had high levels of circulating IC (2-40 muEq/ml). Six BAL specimens (26%) also contained IC. The estimated level of IC in lung epithelial lining fluid (after correcting for dilution) was up to fivefold higher than IC concentration in corresponding sera. Specific antibodies to HIV-1 were demonstrated in 35% of the BAL samples by ELISA and in 65% by Western blotting.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Characterization of cells, immunoglobulins, and immune complexes present in the bronchoalveolar lavage of pediatric AIDS patients. 157 Dec 28

We reported here a case of pure white cell aplasia (PWCA). A 23-year-old man was admitted to our hospital in September 1989 because of agranulocytosis, fever, and anal pain. He had no history of toxic-drug exposure or blood transfusion. Laboratory studies were all within the normal range except white blood cell count of 2,300/microliters with no neutrophils and low serum IgA level (28 mg/dl). Bone marrow examination showed hypocellular with erythroid predominance and no granulocyte maturation beyond the myelocyte. Complement-dependent suppression of autologous and heterologous granulocyte-macrophage colony-forming units by the patient's serum could be demonstrated. Though corticosteroid administration was ineffective, neutropenia improved by plasmapheresis. Furthermore, recombinant granulocyte colony stimulating factor (rG-CSF) could release him from persistent bacterial infection of anal fistula by transient improvement of neutropenia. These findings suggest a humoral autoimmune mechanism for the pathogenesis of PWCA and the effectiveness of rG-CSF for the patient with severe infections.
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PMID:[Pure white cell aplasia (PWCA) with an inhibitor against colony-forming unit of granulocyte-macrophage (CFU-GM)]. 170 3

We prospectively studied newborn infants with sepsis and neutropenia who were randomly selected to receive standard supportive care and either adjuvant granulocyte transfusions or intravenous immune globulin (IVIG) infusions; 21 infants received granulocyte transfusions and 14 received IVIG infusions. Half of the patients were premature (gestational age less than or equal to 32 weeks); the average postnatal age was 5 days (range 3 to 8 days). All infants had neutropenia by the criteria of Manroe et al., and the mean average bone marrow neutrophil storage pool ranged between 35% and 37%. There were no significant differences with respect to serum IgG, IgA, IgM, and total hemolytic complement values between treatment groups or between survivors and nonsurvivors. Clinical severity as defined by hypoxia, acidosis, and hypotension was similar between treatment groups. Group B streptococcus was the most common organism identified and accounted for almost 33% of all bacterial isolates. There was a significantly different survival rate in the group receiving polymorphonuclear leukocyte transfusions (100%, 21/21) compared with the group receiving IVIG infusions (64%, 9/14; p = less than 0.03). There were no significant complications in either treatment group with respect to fluid overload, secondary infection, blood group sensitization, pulmonary complications, or graft-versus-host disease. This pilot study suggests a possible benefit of granulocyte transfusions compared with 'IVIG therapy in the adjuvant treatment of neonatal neutropenia and overwhelming bacterial sepsis.
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PMID:Randomized trial of granulocyte transfusions versus intravenous immune globulin therapy for neonatal neutropenia and sepsis. 151 35


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