Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A deficient erythrocyte pyruvate kinase observed in a patient with congenital non-spherocytic anaemia was characterized by the following properties: very low activity in haemolysates, decreased thermal stability, slightly increased urea denaturation, high affinity for PEP, poor FDP activation, normal ATP inhibition, decreased affinity for ADP, normal pH of optimal activity, and presence of an abnormal slow-moving component in this layer polyacrylamide gel electrophoresis. The patient was probably double heterozygous for two different deficient mutants of erythrocyte pyruvate kinase.
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PMID:A deficient pyruvate kinase with an electrophoretically slow-moving component. 1 93

Platelet functions and blood coagulation have been regularly investigated in 31 patients undergoing maintenance haemodialysis for 5 months to 6 years. Fifteen of them suffered from at least two arteriovenous fistula thrombosis during the year prior the first examination. Eleven patients, including eight with recurrent thrombosis, received 300-400 mg per day dipyridamole during 1 month to 2 years. Some abnormalities are commonly observed in the whole studied population: lowering of platelet adhesiveness, defective aggregation in the presence of both collagen and ADP 5. 10-5 M; increased level of factor V and mainly factor VIII. Mean platelet factor 3 activity was in the normal range with variations from one case to another. The only unusual feature observed in patients with recurrent thrombosis was an increase of platelet aggregation induced by ADP 0.5. 10-6 M. Neither spontaneous aggregation nor significant abnormality of plasminogen level and plasma antithrombin activity were observed. Under dipyridamole therapy, correction of platelet hyperaggregability was observed in all patients and improvement of platelet adhesiveness in half the studied cases (despite the unchanged anaemia). The treatment significantly decreased the frequency of arteriovnous fistule thrombosis in the six patients observed during two consecutive years, the first one without and the second under treatment: the total number of thrombosis was 18 during the first and 3 in the second period.
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PMID:[Disorders of haemostasis in patients undergoing maintenance haemodialysis, with special reference to recurrent arteriovenous fistula thrombosis. Effects of dipyridamole (author's transl)]. 82 31

Pentamidine isethionate is a trypanocidal drug used for the treatment of Pneumocystis carinii pneumonitis. Hematological complications have occasionally been reported and include anemia, leukopenia, and thrombocytopenia. We report here several qualitative abnormalities of in vitro platelet function and coagulation that have not been described previously. Platelets were exposed in vitro to concentrations of pentamidine isethionate ranging from 0.5 to 100 mug/ml of platelet-rich plasma. Clot retraction, platelet adhesiveness to glass beads, and platelet aggregation (adenosine 5'-diphosphate [ADP], thrombin, epinephrine, collagen, and ristocetin) were inhibited in a dose-dependent fashion. The addition of pentamidine isethionate after aggregation had been initiated with ADP reversed both primary and, to a lesser degree, secondary aggregation. Platelet factor 3 availability and serotonin uptake and release (using collagen as the releasing agent) were not inhibited. Serotonin release with 10(-4) M ADP was slightly inhibited. Pentamidine isethionate prolonged the thrombin time of plasma at concentrations of 5 mug/ml and greater. The prothrombin time was prolonged at concentrations greater than 10 mug/ml of plasma. The inhibition of aggregation was reversed by washing and resuspension in plasma or by the addition of calcium or magnesium ions.
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PMID:In vitro inhibition of platelet function and coagulation by pentamidine isethionate. 92 Dec 38

The following phosphate compounds of the erythrocyte acid-soluble fraction were subjected to chromatographic separation: ADP, ATP, adenylo-diphosphoglycerate, 2,3-diphosphoglycerate, hexose monophosphate, hexose diphosphate. In each of the fractions total phosphorus, and in fraction II inorganic phosphorus, were estimated. The material was derived from ten newborns with haemolytic disease as a result of ABO incompatability and from ten full-term healthy newborns, just after birth. The concentration of the compounds assayed, except for 2,3-DPG (the values in both groups were similar) was higher in the erythrocytes from affected newborns, but lower than that found in the material derived from the newborns with Rh incompatibility. It is suggested that the metabolism of erythrocytes of the newborns with haemolytic ABO disease may be somewhat different from that in Rh incompatibility cases because in the former the haemolysis is weaker, the anaemia is less pronounced and the tissue hypoxia is of a smaller degree.
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PMID:Phosphate compounds in the erythrocytes of newborns with ABO systems haemolytic disease. 103 90

Four new red-cell pyruvate kinase (PK) variants are presented along with one case of so-called classical type PK deficiency. PK 'Tokyo II' had a low activity, Km (PEP) and Vmax, but a normal urea stability and only slight deviation from normal in neutralization tests by antiserum. It had a normal nucleotide specificity, abnormal electrophoretic mobility (fast moving) and the variant was associated with a mild hemolytic anaemia. PK 'Maebashi' had a low activity, high Km (PEP), low Vmax, urea instability, decreased reactivity to antiserum, normal electrophoretic mobility, normal nucleotide specificity and was associated with a moderate haemolytic anaemia. PK 'Tsukiji' had low activity, high Km (PEP), markedly high Vmax, urea instability, decreased reactivity to antiserum, abnormal electrophoretic mobility (fast moving) and grossly abnormal nucleotide specificity especially abnormal behaviour to ADP. The haemolytic process in this case was moderate to severe. PK 'Ube' was electrophoretically abnormal (fast moving) but otherwise had normal characteristics and the propositus was healthy and not anaemic. PK 'Ube' was found by electrophoretic screening for genetic PK polymorphism. In the classical type PK deficiency, the usual red-cell PK (PK-R1 and PK-R2) was not demonstrable by electrophoresis but instead M2-type PK was present, presumably by compensatory process. Kinetic studies confirmed that the patient's red-cell PK consisted of M2-type PK. This patient had a severe haemolytic anaemia.
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PMID:Four new pyruvate kinase (PK) variants and a classical PK deficiency. 120 Nov 98

Hematograms, platelet function, and blood-enzyme chemistry were compared in two similar saturation-excursion dives, one conducted in a hyperbaric chamber and the other in the open sea. The chamber dive was more stressful in that it was preceded by a series of bounce decompression dives (one of which produced a 100% incidence of cutaneous pruritus in four subjects) and in that the excursions from saturation depth (60 fsw or 2.818 ATA) were longer and deeper (producing one case of O2 convulsions, one of confirmed decompression sickness, and several of Doppler-detected vascular bubbles). The chamber dive was associated with a marked and significant reduction in circulating platelet count; significant increases in plasma enzyme activities in the victim of O2 toxicity (LDH, CPK) and in one subject with Doppler bubbles and questionable bends symptoms (LDH, GOT, GPT) but not in another; and mild but significant anemia. In the open-water dive, one subject, who developed symptoms of gastroenteritis, showed moderate elevation of LDH, GOT, and GPT activity. No significant change in platelet counts occurred. Both dives were associated with elevated white-cell counts, apparently as a result of numerous minor infections, and reduced sensitivity of platelets of ADP-induced aggregation.
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PMID:Hematology and blood chemistry in saturation diving: II. Open-sea vs. hyperbaric chamber. 122 83

Patients with uraemia have a defect haemostasis caused by severe anaemia and disturbances of platelet/vessel wall interactions. Recombinant human erythropoietin (rHuEPO) treatment not only corrects anaemia, but also shortens the bleeding time. There are few reports dealing with changes of haemostasis during the first month of rHuEPO treatment. We studied platelet function after 1, 2, 4, 8, and 12 weeks of rHuEPO treatment. Erythropoietin was given to 19 dialysed patients with chronic uraemia in a dose of 2000 u subcutaneously 3 times a week. Bleeding time showed a significant fall as early as after the first week of rHuEPO treatment (p < 0.05). After the first month the bleeding time became normal in most of the patients. A significant rise in ristocetin-induced platelet aggregation was observed from the first week of therapy. It showed a strong correlation with the shortening of the bleeding time. Collagen-induced aggregation followed the same pattern but the changes were not striking. There was not significant difference in platelet adhesion, platelet aggregation in the whole blood and those induced by ADP and arachidonic acid. Platelet serotonin concentration was also showed to increase during rHuEPO therapy. We conclude that rHuEPO improves haemostasis by influencing platelet aggregation possibly involving a serotoninergic mechanism but on the other hand may increase a tendency to thrombosis.
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PMID:[Platelet aggregation during the recombinant human erythropoietin (rHuEPO) treatment of patients with uremia]. 148 23

Patient A.F. is a 28-year-old polytransfused woman with an inherited bleeding disorder, Glanzmann's thrombasthenia. An abnormal platelet function is linked to severe decreases in the platelet content of the integrins GP IIb and GP IIIa. In 1987 the patient gave birth to a child with severe anemia and thrombocytopenia. Serological tests revealed the presence of anti-platelet antibody together with an anti-Rhesus D. Western blotting identified a major antibody that reacted with a protein of 90-95 kDa present in platelets and endothelial cells. This was identified as the beta 3 integrin subunit (GP IIIa). Antibody-binding required intact disulfides, while controlled digestion with proteases showed the determinant(s) to be retained within chymotrypsin- (50, 63 kDa) and Staphylococcus aureus V8 protease-derived (25-38 kDa) fragments of GP IIIa. Direct binding assays performed in the presence of monoclonal antibodies specific for different epitopes on GP IIb-IIIa complexes confirmed that the epitope was exposed on intact platelets and revealed a specific inhibition of A.F. IgG binding by the monoclonal antibody, AP-3. Other tests confirmed that the antibody reacted independently of the PlA or Pen polymorphisms carried by GP IIIa. IgG purified from A.F. plasma by adsorption and elution from paraformaldehyde-fixed normal platelets or electrophoretically separated GP IIIa was an inhibitor of ADP-induced platelet aggregation. Unexpectedly, Western blotting showed trace amounts of abnormally migrating GP IIIa in A.F. platelets, which retained an ability to react with her antibody. This suggests that the patient has formed an autoantibody reactive with an active site of the beta 3 integrin subunit and linked to the development of neonatal thrombocytopenia.
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PMID:Characterization of an antibody to the integrin beta 3 subunit (GP IIIa) from a patient with neonatal thrombocytopenia and an inherited deficiency of GP IIb-IIIa complexes in platelets (Glanzmann's thrombasthenia). 163 70

Hemostatic measurements were undertaken in eight chronic hemodialysis uremic patients on recombinant human erythropoietin (rHuEpo). Same measurements were repeated in another seven patients in whom anemia was corrected by the transfusion of red blood cells. The correction of the anemia by rHuEpo therapy was accompanied by 1. correction of the prolonged Simplate Bleeding Time (BT) to normal less than 10.0, minutes after 16 weeks of rHuEpo treatment; 2. significant increases in the levels of fibrinogen, clotting FVIII:C, vWF:antigen, vWF:ristocetin cofactor and platelet count; 3. enhanced aggregation responses to ADP, adrenaline, arachidonic acid, collagen and ristocetin. There was no significant fluctuation in other coagulation parameters PT, APTT, TT, reptilase time and antithrombin III and plasma fibrinogen. In patients who were treated with RBC transfusion and despite the correction of the anemia, the bleeding time shortened significantly but not corrected, mean BT before and after RBC transfusion was 17.1 +/- 1.4 and 11.6 +/- 1.9 minutes respectively. Besides there was significant elevation of vWF:Ricofactor levels but not FVIII:C, vWF:Ag or platelet count. Platelet aggregation responses to ADP remained unchanged. It is concluded that significant elevations of FVIII:related activities, plasma fibrinogen, improved platelet aggregability and correction of the BT are salient hemostatic changes that follow rHuEpo therapy in uremic patients.
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PMID:Effect of recombinant human erythropoietin (rHuEpo) on the hemostatic system in chronic hemodialysis patients. 175 76

Erythrocytes are known to influence hemostasis. Bleeding times are prolonged in anemia and corrected by normalizing the hematocrit. We now demonstrate that intact erythrocytes modulate biochemical and functional responsiveness of activated platelets. A two-stage procedure, permitting studies of cell-cell interactions and independently evaluating platelet activation and recruitment within 1 min of stimulation, was developed. Erythrocytes increased platelet serotonin release despite aspirin treatment, enzymatic adenosine diphosphate removal, protease inhibition, or combinations thereof. The data suggested that erythrocyte enhancement of platelet reactivity can reduce the therapeutic effectiveness of aspirin. Erythrocytes metabolically modified platelet arachidonate or eicosapentaenoate release and eicosanoid formation. They promoted significant increases in cyclooxygenase and lipoxygenase metabolites upon platelet stimulation with collagen or thrombin. However, with ionophore, erythrocytes strongly reduced platelet lipoxygenation. These erythrocyte modulatory effects were stimulus-specific. Activated platelet-erythrocyte mixtures, with or without aspirin, promoted 3-10-fold increases in extracellular free fatty acid, which would be available for transcellular metabolism. Erythrocyte-induced increases in free eicosapentaenoate may contribute to antithrombotic and anti-inflammatory effects of this fish oil derivative. These results provide biochemical insight into erythrocyte contributions to thrombosis and hemostasis, and support the concept of thrombus formation as a multicellular event.
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PMID:Enhancement of platelet reactivity and modulation of eicosanoid production by intact erythrocytes. A new approach to platelet activation and recruitment. 199 40


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