Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 23-year-old Japanese male with severe lead intoxication accompanied by hemolytic anemia was studied. The patient had taken 12 g to lead in about a month. He had moderate hemolytic anemia (Hb 8.9 g/100 ml) with reticulocytosis ranging from 2.5 to 11.7%. Peripheral blood smear showed nucleated red cells (42/200 white blood cells) and marked basophilic stippling in the red cells. Activities of erythrocyte enzymes were either normal or increased except for pyrimidine 5'-nucleotidase (P5N) and delta-aminolevulinic acid dehydratase (ALA-D) both of which were found to be decreased, being 48.8% and 4.1% of the normal controls respectively. Erythrocyte reduced glutathione (GSH) was high (145.6 mg/100 ml RBC). Erythrocyte pyrimidine nucleotides were accumulated up to 10.2% of total nucleotides. The level of lead in peripheral blood was 112 microgram per 100 ml blood. Ca2+-Na2-EDTA was given to the patient as treatment. The level of lead in the blood decreased gradually and hemolytic anemia improved. Basophilic stippling in the red cells disappeared. These results confirmed the findings of Valentine et al [1] that lead-induced deficiency of P5N resulted in basophilic stippling and hemolytic anemia just like hereditary hemolytic anemia due to P5N deficiency.
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PMID:A case of lead intoxication: clinical and biochemical studies. 626 39

The activity of 18 red blood cell (RBC) enzymes and reduced glutathione (GSH) content were measured in 70 normal subjects, in 50 heterozygous beta-thalassaemia carriers and in 50 non-thalassaemic patients with haemolytic anaemia and high reticulocyte counts. In addition, pyrimidine 5'nucleotidase (P5N) activity was also determined in 34 patients with hypochromic, microcytic, iron deficiency anaemia. beta-Thalassaemia trait was associated with an increase in almost all of the enzyme activities, except for 2,3-bisphosphoglycerate synthetase (BPGS) and glutathione reductase (GR) which were normal and for acetylcholinesterase (AChE) and P5N which were slightly and markedly decreased respectively. The increases in enzyme activities were similar to those observed in patients with non-thalassaemic reticulocytosis except for glyceraldehydephosphate dehydrogenase (GAPD), phosphoglyceratekinase (PGK), pyruvate kinase (PK), glutathione peroxidase (GPX) and adenylate kinase (AK) which were higher than in non-thalassaemic group of patients with increased number of reticulocytes. No correlation was found between the severity of P5N deficiency and the intensity of basophilic stippling which was present in 46 of 50 thalassaemic carriers here studied. In addition, GSH content and UV absorption spectra of deproteinized thalassaemic RBC extracts were also found to be normal. The present findings provide further information on the metabolic status of RBC in beta-thalassaemia trait and suggest a possible molecular explanation for the frequently observed basophilic stippling in this disease.
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PMID:Pyrimidine 5'nucleotidase and several other red cell enzyme activities in beta-thalassaemia trait. 632 Aug 62

2,3 dihydro-IH-imidazo (1,2-b) pyrazole (IMPY) is a potential chemotherapeutic agent known to inhibit cellular DNA synthesis by blocking ribonucleotide reductase. During Phase I clinical studies with IMPY, patients developed a dose dependent hemolytic anemia possibly secondary to oxidant damage to the RBC. We therefore studied the effect of IMPY on the metabolism of human RBC's in vitro. IMPY, in clinically achievable concentrations, stimulated the hexose monophosphate shunt (HMPS) pathway of RBC's. This was associated with the generation of reactive oxygen species as demonstrated by the glutathione (GSH) instability and enhanced formate oxidation of RBC's incubated with the drug. In addition, the GSH concentration of the red cells of a patient fell during a continuous infusion of IMPY. These effects of IMPY on red cell metabolism in vitro and in vivo are similar to those of drugs known to cause oxidative damage to this cell. The capacity of IMPY to act as an oxidant could explain the hemolytic anemia seen in patients receiving this drug.
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PMID:Effect of 2,3-dihydro-1H-imidazo [1,2-b]pyrazole (IMPY) on the metabolism of human red cells. 667 73

The clinical and laboratory features of a 3-mo-old black male infant with glutathione (GSH) synthetase deficiency of the generalized type was evaluated. Partial albinism, brisk hemolytic anemia, recurrent febrile episodes, and mental retardation were noted. Also, severe recurrent metabolic acidosis and marked oxoprolinemia and oxoprolinuria were found in the proband but not in his first-degree relatives. The relationship of these disease manifestations to the underlying metabolic defect is discussed.
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PMID:Hemolytic anemia, recurrent metabolic acidosis, and incomplete albinism associated with glutathione synthetase deficiency. 688 23

1. G6PD mutants with CHD have decreased GSH, despite reticulocytosis, and increased membrane polypeptide aggregates. Aggregates increase logarithmically with decrease in RBC GSH. 2. These aggregates contain spectrin and can be depolymerized by disulfide reducing agents. Disulfide bonds between spectrin molecules and between the cytoskeleton and the cytoplasmic protein rigidify the red cell membrane and decrease RBC survival. 3. Direct oxidative damage of the RBC membrane, not Heinz body formation, explains the hemolytic anemia of G6PD mutants with CHD. This membrane damage may constitute a useful model system of oxidant-induced injury of other cells, and is an example of postsynthetic modification of membrane proteins by a nonmembrane gene.
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PMID:Erythrocyte membrane protein changes in glucose-6-phosphate dehydrogenase mutants with chronic hemolytic disease: an example of postsynthetic modification of membrane proteins. 715 68

Xylitol was investigated for its ability to ameliorate hemolytic anemia induced by acetylphenylhydrazine in rabbits. Animal experiments were performed using two different concentrations of xylitol, a 5% and a 10% solution with a total dose of 2 g/kg body weight and infusion rates of 10 mg and 20 mg xylitol per kg body weight per minute respectively. Two doses of acetylphenylhydrazine (APH), 5 and 10 mg per kg, were injected intraperitoneally as hemolytic inducers in different groups of rabbits. All the rabbits infused with xylitol showed significantly less acute APH-induced hemolysis. The isotonic 5% xylitol solution was found to maintain and restore the hematological parameters (packed cell volume, hemoglobin concentration, reduced glutathione (GSH) content, and reticulocyte counts) better than the 10% xylitol solution. Increased 51CR-red cell survival confirmed the beneficial effect of xylitol. The survival of erythrocytes as represented by chromium-labeling in rabbits infused with 5% xylitol after treatment with 10 mg/kg APH increased from about 33% (the survival of red cells in rabbits injected with APH alone) to 67% of normal rabbits' red cell survival. Erythrocytes in APH-treated animals took up xylitol more readily than erythrocytes from control animals. Our results in rabbits suggest that (1) non-toxic dosage of xylitol is effective in ameliorating the hemolytic episode induced by APH, (2) there is a dose relationship between the hemolytic effect induced by APH and the preventive effect offered by xylitol, (3) drug-challenged cells effectively acquired two to three fold more xylitol to compensate for the cellular needs than that of the normal cells, and (4) sufficient xylitol (55 mg/dl) to act as substrate for xylitol dehydrogenase was recovered intracellularly in drug-challenged rabbit erythrocyte in vivo, in spite of a low plasma (less than 30 mg/dl) concentration of the substrate. This antihemolytic affect of xylitol is likely accomplished through NADPH generation, which maintains the level of GSH and protects the hemoglobin and other structural and functional proteins against peroxidative damage.
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PMID:Xylitol mediated amelioration of acetylphenylhydrazine-induced hemolysis in rabbits. 728 79

The still unexplained occurrence of hemolytic anemia in cases of alcoholic liver disease prompted us to study the toxic effect of ethanol on the membrane and metabolic properties of erythrocytes (RBC) in chronic alcoholics (n = 7). Raised susceptibility of RBC to hydrogen peroxide with significantly increased fluidity of membrane lipids were obtained signaling an oxidative metabolic stress. A twofold reduction of ATP and GSH initiated an oxidation of cellular sulfhydryl groups thus impairing enzyme kinetics of pyruvate kinase (PK). Yet heat stability of PK was markedly reduced only in those fractions containing older RBC. Further results indicate that ethanol-induced acquired PK instability may be triggered by a dialyzable and hence non-protein-bound compound, not detectable by experimental procedures owing to its low molecular weight. Thus it is conceivable that the hemolytic implication of ethanol in alcoholic liver disease may be due to abnormal membrane fluidity and metabolic disorder finally involving PK-control mechanism.
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PMID:Erythrocyte membrane fluidity, lipid peroxidation and lysis in alcoholic liver disease. 731 5

Although humans may accept fairly large amounts of orally ingested copper (0.25 to 1.0 gm) without visible harmful effects, patients with Wilson's disease, and persons with G6PD deficiency may represent persons at unusual risk to hemolytic anemia from ingestion of Cu(II). This study reports that in vitro exposure of G6PD deficient red blood cells to copper produced marked elevations of methemoglobin and decreases in GSH when compared with normal red cells. Chlorite, a by-product of chlorine dioxide disinfection of water, produced decreases in GSH and G6PD activity, while increasing methemoglobin levels markedly over red cells with normal G6PD activity. The combined action of chlorite and copper was additive in producing increased levels of hemoglobin and decreases in levels of GSH and G6PD deficient cells. The combined ingestion of copper and chlorite may represent an increased risk to persons with G6PD deficiency.
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PMID:G6PD-deficiency: a potential high-risk group to copper and chlorite ingestion. 746 5

Chlorite, a by-product of chlorine dioxide disinfection of water, is a strong oxidant compound that produces markedly exaggerated effects in vitro on red cells of G6PD deficient humans when compared to normal human cells. Levels of methemoglobin are significantly greater and GSH levels significantly lower in the G6PD deficient cells than in normal cells after chlorite exposure. Persons with G6PD deficiency may be 3 to 4 times more likely to develop hemolytic anemia from chlorite exposure as persons with normal activity levels when GSH levels are used as a measure of susceptibility. The proposed use of chlorine dioxide as an alternate disinfectant for drinking water supplies should consider this potential high risk group.
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PMID:Groups at potentially high risk from chlorine dioxide treated water. 746 14

Changes in red blood cell (RBC) lipid peroxidation [measured by malonyl dialdehyde (MDA) concentration], glutathione (GSH) metabolism, antioxidant enzyme activities (catalase, superoxide dismutase, glutathione peroxidase) and haemoglobin (Hb) metabolites (metHb, carboxy Hb) were studied in six children with post-enteropathic (D+) haemolytic uraemic syndrome (HUS) and ten controls. The in vitro effect of hydrogen peroxide [acetylphenylhydrazine (APH) test] on GSH and Hb metabolism was also investigated. MDA levels were significantly higher and the antioxidant enzyme activities were lower in HUS patients than in the controls (P < 0.01). The oxidised glutathione concentration was significantly higher in the patients than in the control children (26.3 +/- 12.6 vs. 10.9 +/- 1.8 nmol/g Hb. Percentage values of carboxy Hb and metHb were also higher in HUS (P < 0.01). Incubation of RBC with APH induced a more pronounced decrease in the concentration of GSH (P < 0.001) and a significant increase (P < 0.01) in the level of metHb and carboxy Hb in the HUS patients. This suggests that there is reduced RBC GSH stability in HUS. Utilisation of GSH and antioxidant enzymes leads to increased Hb oxidation and haemolysis. The oxidative damage may have an important role in the pathogenesis of haemolytic anaemia in HUS.
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PMID:Oxidative damage of red blood cells in haemolytic uraemic syndrome. 814 20


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