Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Some biochemical, pathohistological and ultrastructural changes in liver after the action of single intraperitoneal administration of furosemide (Furantryl-Pharmachim) in a dose of 300 mg/kg of body mass were studied in male white rats of Wistar strain. The results from the performed experiments established increased activity of serum enzymes GOT, GPT, GGT, alkaline phosphatase as well as low values of serum cholinesterase. Pathohistologic and electron microscopy examination discovered liver damage with typical congestive changes mainly--manifested local erythremia and a reduced fluid content of the blood in the liver with blockage in the sinusoidal pole of hepatocytes; there were also focal micronecrosis, considerable reduction of glycogen and slight centrolobular steatosis. The possibility is discussed for usage of hepatotoxicity, induced by furosemide, in examining the effects of some drugs with potential hepatoprotective activity.
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PMID:[Experimental liver damage by furosemide for studying drugs with hepato-protective activity]. 280 81

From 1963 to 1983, I treated 100 patients with polycythemia vera, using phlebotomy and the adjunctive agent hydroxyurea. These 78 male and 22 female patients ranged in age from 24 to 88 years (mean 55.7). Duration of therapy ranged from three to 216 months (mean 64.9). The mean daily dose was 0.72 gm, and the median dose was 0.64 gm. Hydroxyurea gave adequate control of red cells, platelets, and spleen size. Cytopenia was not observed. Phlebotomy requirements were markedly reduced. Leukocyte alkaline phosphatase scores were generally lowered and several blood chemistry values returned to normal. Side effects were minimal, and there were no drug-related deaths. Infections were not a problem. Hydroxyurea, a metabolic inhibitor of desoxyribonucleic acid, does not interfere with the synthesis of ribonucleic acid or protein and is thus probably less leukemogenic than radioactive phosphorus and alkylating agents. Acute myelogenous leukemia was seen in one patient after five years of continuous hydroxyurea therapy. He had received no other myelosuppressant agent. Because hydroxyurea is safe and effective in the treatment of polycythemia vera, it should be considered as first-line therapy. It probably offers practical and theoretic advantages over present therapy particularly when the disease is not well controlled by phlebotomy alone.
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PMID:Hydroxyurea in the treatment of polycythemia vera: a prospective study of 100 patients over a 20-year period. 382 16

To characterize the biological changes which result in increased granulocyte alkaline p-nitrophenyl phosphatase activity in patients with polycythemia vera, the enzyme was purified from granule fractions of sucrose homogenates made from dextran-sedimented leukocytes of normal subjects and patients with polycythemia vera. Polycythemic blood yielded 3-10 times as much granulocyte alkaline phosphatase per 10(9) leukocytes as did normal blood. Sodium dodecyl sulfate extracts of granules were purified by DEAE-cellulose chromatography and sucrose gradient centrifugation to apparent homogeneity as judged by polycarylamide disk gel electrophoresis. Granulocyte alkaline phosphatase from normal subjects was purified 6910-fold with a 60% yield and a specific activity of 47 U/mg. Granulocyte alkaline phosphatase from polycythemic patients was purified 1.166-fold with a 50% yield and a specific activity of 70 U/mg. The two enzymes did not differ in molecular weight; both appeared to be about 160,000 daltons by sucrose gradient centrifugation. Both appeared to be zinc metalloenzymes, in that they were specifically inhibited by o-phenanthroline. Their elution requirements when adsorbed to DEAE-cellulose suggested they were lipoproteins although the content of phosphorus was below the threshold of detection. The identity of the two enzymes was suggested by immunological studies in which antibody prepared against purified polycythemia vera enzyme gave a precipitation reaction of identity with another polycythemia vera enzyme and two pools of normal enzyme. It is possible to account for the difference in alkaline phosphatase activity between the granulocytes of patients with polycythemia vera and normal subjects by differences in the quantity of enzyme synthesized.
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PMID:Granulocyte alkaline phosphatase. Studies of purified enzymes from normal subjects and patients with polycythemia vera. 473 97

Symptomatic erythrocytosis developed in a 59-year-old man with polycystic kidney disease (PKD) while he was receiving maintenance hemodialysis. Major clinical and laboratory data suggested a diagnosis of polycythemia vera (PV), despite a normal serum alkaline phosphatase level and leukocyte count. Secondary erythrocytosis, related to chronic hypoxemia and increased erythropoietin production, was excluded by appropriate laboratory studies. Despite previous documentation of secondary erythrocytosis in patients receiving hemodialysis, to my knowledge, PV has not been described in this population.
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PMID:Polycystic kidney disease and polycythemia vera. Occurrence in a patient receiving hemodialysis. 661 11

We describe two patients who had similar features of erythrocytosis and marked leukocytosis but who were subsequently classified as having chronic myelogenous leukemia and polycythemia vera, respectively, using leukocyte alkaline phosphatase scores and cytogenetic studies to detect the Philadelphia chromosome. The patient diagnosed as having polycythemia vera had a leukocyte count of 164,000/cu mm, which remained at similar levels when the patient was not in remission. We believe this is the first well documented case of PV with a leukocyte count above 150,000/cu mm.
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PMID:Erythrocytosis and marked leukocytosis in overlapping myeloproliferative diseases. 695 6

In hematological diseases such as myeloproliferative disorders (MPD) or myelodysplastic syndromes (MDS), some abnormalities in the chemiluminescence of neutrophils are observed. There are two groups; one includes chronic myelogenous leukemia (CML), essential thrombocythemia (ET) and MDS, which all have decreased chemiluminescence of neutrophils. The other group includes polycythemia vera (PV) which has increased neutrophil chemiluminescence. We studied the neutrophil function by analyzing the chemiluminescence in 35 patients with hematological diseases. In most of these cases the defects in chemiluminescence in 35 patients with hematological diseases. In most of these cases the defects in chemiluminescence in response to N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP) were correlated with those in response to phorbol 12-myristate 13-acetate (PMA). But there were exceptional cases in which the maximal light emission of chemiluminescence (Max CL) in response to FMLP was obviously lower than controls despite the fact that the Max CL in response to PMA was the same as the controls. These facts suggest a heterogenicity of the defect site in these diseases. There was a correlation between the level of chemiluminescence and the neutrophil alkaline phosphatase (NAP) activity in these patients. In vitro culture of CML neutrophils with granulocyte colony-stimulating factor (G-CSF) showed a correlation between the increase in the level of chemiluminescence and NAP activity. These results suggest that NAP may take part in the control of neutrophil function.
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PMID:Chemiluminescence of neutrophils in patients with myeloproliferative or myelodysplastic hematologic diseases--relation to neutrophil alkaline phosphatase activity. 768 68

A few simple, low-priced, outpatient investigations, including an upper abdominal ultrasonography, a measurement of the arterial oxygen saturation and, in some cases, a leukocyte alkaline phosphatase score (LAP) suffice to find out the etiology of an increased red cell mass in most patients. The diagnosis of polycythemia vera (PV) could be accepted in patients with an increased red cell mass if the spleen is enlarged and if the arterial oxygen saturation is normal, although the latter measurement may not be required in typical cases. If the spleen is not enlarged, the diagnosis of PV could be accepted if, in addition, the leukocyte or thrombocyte count is increased. An elevated LAP score also points to the diagnosis of PV, provided fever or inflammation are not present. If, at that stage, an etiologic diagnosis has not been made, smoker's polycythemia should be considered and excluded. The next step should include a serum erythropoietin assay and culture of erythroid stem cells before performing investigations aimed at ruling out the many conditions associated with secondary polycythemia.
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PMID:Biological and radiological investigations in patients with an increased red blood cell mass: Which are needed? Which are useful? which are unnecessary? 803 33

Using flow cytometry, we quantitatively examined the density of the CD16 (IgG Fc receptor III) antigen on neutrophils in healthy control subjects, in patients with neutrophilia due to bacterial infection, and in patients with chronic myeloproliferative disorders (chronic myeloid leukemia [CML], polycythemia vera, or essential thrombocythemia). The density was expressed as the mean fluorescence intensity of neutrophils stained with fluorescein isothiocyanate-labeled anti-CD16 monoclonal antibody. We also determined leukocyte alkaline phosphatase activity semiquantitatively in the same population. The mean (+/- SD) density of the CD16 antigen on neutrophils in patients with CML (n = 13; 240.4 +/- 134.8) was lower (P<.001 ) than in healthy control subjects (n = 25; 656.6 +/- 238.0), and the density was also lower than in patients with bacterial infection (n = 15; 671.5 +/- 288.1), polycythemia vera (n = 7; 552.6 +/- 99.9), or essential thrombocythemia (n = 11; 671.5 +/- 411.5). The density of the CD16 antigen was 300 or more in all healthy control subjects and in all patients examined, except for those with CML. The CD16 antigen density was less than 300 in 10 of the 13 patients with CML. Leukocyte alkaline phosphatase activity was also low in 10 of the 13 patients with CML. These findings indicate that flow cytometric analysis of the density of neutrophil CD16 antigen is useful for the differential diagnosis of CML from other chronic myeloproliferative disorders.
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PMID:CD16 antigen density on neutrophils in chronic myeloproliferative disorders. 953 1

This article presents a case of 45-year-old man with polycythemia vera non diagnosed before. The first symptom of polycythemia vera was acute congestive heart failure which suggested diagnosis of myocarditis. Polycythemia vera was confirmed by raised hematocrit, significantly increased platelet count, normal oxygen saturation, score for leukocyte alkaline phosphatase (LAP)-130 and splemomegaly. Echocardiography revealed left ventricular histological. Coronary arteriography showed normal coronary arteries. Finding of histological examination of the endomyocardial biopsy were described as necrosis of myocytes and abnormal blood flow in very small coronary vessels. It was the main reason of dilated cardiomyopathy caused by microinfarcts in polycythemia vera. Hematological parameters were reduced to normal levels after hydroxyurea treatment. Digitalis and ACE-inhibitor therapy quickly improved cardiovascular status from III to II NYHA class.
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PMID:[A case of dilated cardiomyopathy caused by myocardial microinfarcts in the course of polycythemia vera]. 958 43

Expression of alkaline phosphatase (ALP) on the surface membrane of neutrophils (mNAP) was studied by immunofluorescence using an anti-ALP monoclonal antibody. Fluorescent intensity distribution of mNAP was analyzed using FACS (fluorescence-activated cell sorter). The mean fluorescent intensity (MFI) of the mNAP in this assay was well correlated with the neutrophil ALP (NAP) score demonstrated cytochemically (r = 0.832). mNAP levels in various hematological disorders were evaluated by % mNAP+ cells and MFI. The levels in aplastic anemia and polycythemia vera were significantly higher, and in chronic myelocytic leukemia and paroxysmal nocturnal hemoglobinuria (PNH), the levels were significantly lower compared with the levels in healthy volunteers. Two-color immunofluorescence with anti-ALP and anti-CD16 showed that the PNH clone was essentially negative for mNAP, whereas residual normal neutrophils (CD16+) had levels slightly higher than those in normal individuals. Highly reproducible results were obtained in the blood samples which were stored at 4 degrees C for at least 24 hr without any treatment prior to immunofluorescent staining. No degradation of fluorescent intensity was seen 4 days after staining and fixation. The mNAP assay is simple, without subjective evaluation for quantification, and is useful for differential diagnosis of hematological disorders.
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PMID:Assessment of alkaline phosphatase on the surface membrane of neutrophils by immunofluorescence. 988


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