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)

The Effects of Various Substances in the Culture Microenvironment on the Proliferation of Erythroblasts in vitro were Studied and Evaluated by differential cell and mitosis counting in normal and pathologic bone marrow. The mitotic frequency and maturation correlated directly with the erythropoietin content of the medium and were potentiated on addition of either folic acid, etiocholanolon or cAMP. However, erythroblast proliferation was stimulated independently of erythropoietin concentration when either cobalt, and androgen or an anabolic substance was added to the medium. An iron-deficient medium prevented the maturation of erythroblasts to reticulocytes, thereby rendering erythropoiesis ineffective. The existence of an erythrocyte chalone ineffective. The existence of an erythrocyte chalone or an erythropoietin inhibitor could not be deduced from our experiments since the transformation of pluripotent to erythropoetin-sensitive stem cells is not included. A stimulation of the granulopoietic proliferation pool occurred when serum from patients with either polycythemia vera or after acute blood loss was added to the medium.
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PMID:[Regulation of the human erythropoietic proliferation pool by the microenvironment (author's transl)]. 20 43

A high RBC count combined with a low mean volume generally is attributed to thalassemia minor, either alpha or beta, or to polycythemia vera with iron deficiency. Among 330 patients with a mean corpuscular volume (MCV) less than 70 cumu, 35 had increased RBC counts. Of these, 26 had thalassemia minor and four had polycythemia vera. Five had secondary polycythemia (four from hypoxia, one from hypernephroma) with incidental iron deficiency. In the four of these patients given iron, the RBC count remained above normal and the MCV rose to normal. The RBC size distribution curves reliably distinguished between thalassemia minor and polycythemia with iron deficiency.
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PMID:Microcytic polycythemia. Frequency of nonthalassemic causes. 57 67

A method has been developed for the estimation of iron, copper, zinc, calcium and magnesium in human platelets using atomic absorption spectrophotometry. Fe and Cu are either absent or present in very low concentration approaching the limit of detection: Zn, Ca and Mg are readily detectable. EDTA is preferable to ACD(A) or heparin as the anticoagulant for collection in that the platelet yield is reliable: however, slightly low results for Ca and Mg are obtained. Corrections for residual plasma and contaminating red cells are essential. Plasma and platelet metal levels in 33 normal subjects are reported together with the findings in four studies undertaken on platelets obtained from subjects with polycythemia vera.
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PMID:Platelet metal levels in normal subjects determined by atomic absorption spectrophotometry. 58 Sep 87

A patient with classical polycythemia vera (PV) was found to have an inappropriately elevated serum erythropoietin (Ep) level. Investigations did not reveal any lesion or blood abnormality known to be associated with excessive Ep production and erythrocytosis. Sudden withdrawal of blood to reduce the Hb and Hct from 18.5 gm% and 56% to 13.6 gm% and 41.5%, respectively, resulted in an increment of serum Ep to abnormal level. With iron treatment there was a brisk return of Hb and Hct to prebleeding levels which was associated with reduction in the serum Ep. The inverse relationship between the Ep and Hb or Hct is inconsistent with the presence of excessive Ep-producing lesion. These results suggested that the threshold for Ep secretion from normal Ep-secreting tissue to Hb and Hct levels is set at an abnormal level. This patient's marrow cells when cultured in vitro in the absence of Ep, unlike other PV patients' (except one) marrow cells, did not grow erythroid colonies. In the presence of Ep, however, the colonies comparable to those formed from normal marrow cultures were obtained. These results suggested that his marrow erythropoietic cells were neither Ep independent nor Ep-hyperresponsive, as has been suggested by some investigators for erythropoiesis in PV. This patient presents phenomena that hitherto have not been reported.
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PMID:Inappropriate erythropoietin secretion in polycythemia vera. 60 35

The effect of renal failure and bilateral nephrectomy on erythropoiesis and plasma erythropoietic activity was observed in a patient with polycythemia vera. For eight years the patient's hematocrit was maintained between 45 and 50 per cent by phlebotomy and in spite of the development of renal failure the hematocrit did not decline. Following rejection of a renal transplant, the hematocrit fell to 18 per cent but rose to 40 per cent with oral iron therapy. Following bilateral nephrectomy, the hematocrit fell to 29 per cent but subsequently increased to 37 per cent. After an episode of gastrointestinal bleeding the hematocrit was 21 per cent but subsequently rose to 32 per cent. Erythropoietin could not be detected in the plasma either before or after nephrectomy. In addition, erythropoietin failed to stimulate 59Fe incorporation into heme in vitro in the patient's marrow cells. The data incidate that, in polycythemia vera, erythropoiesis does not require erythropoietin.
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PMID:Polycythemia vera in an anephric man. 101 15

Serum iron concentration, serum iron binding capacity and saturation coefficient were assayed in polycythaemia rubra vera and secondary polyglobulia. In the exacerbation stage of polycythaemia rubra, significantly lower Se Fe and SC values were found while in the remission stage and in secondary polyglobulias these values did not differ from those of the normal control.
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PMID:Iron metabolism in polycythaemia rubra vera and secondary polycythaemia. 123 Apr 2

Polycythemia vera (PV) is one of the myeloproliferative diseases, and, as such, is an example of clonal hematopoiesis. The progeny of a single, abnormal, hematopoietic stem cell gain a growth advantage over their normal counterparts resulting in overproduction of red cells generally accompanied by overproduction of granulocytes and platelets as well. There are a variety of nonspecific symptoms at onset related to the increased red cell mass and hematocrit accompanied by the more specific manifestations of pruritus, erythromelalgia, and hepatic, portal, and mesenteric vein thrombosis. Splenomegaly and hypertension are common. The laboratory hallmark is an increased red cell mass. There is also often an increase in white cell count, platelet count, and leukocyte alkaline phosphatase along with other findings reflecting the increased rate of turnover of hematopoietic cells. The bone marrow biopsy generally displays hypercellularity involving all three cell lines and absent iron stores. The diagnosis of PV depends on excluding spurious polycythemia in which there is a high hematocrit but a normal red cell mass and secondary polycythemia in which there is an increased red cell mass in response to tissue hypoxia or the inappropriate production of erythropoietin, generally by a tumor. In addition, one should try to establish the diagnosis in a positive fashion by a combination of studies of the blood and bone marrow. Phlebotomy and occasionally plateletpheresis should be used as acute therapy. Chronic therapy is guided by the knowledge that patients treated with phlebotomy alone have an increased rate of thrombotic complications particularly in older patients and those with previous thrombotic disease. Myelosuppressive therapy can reduce the incidence of these complications, but is commonly associated with an increased incidence of second malignancies, particularly acute leukemia. At present, hydroxyurea is the myelosuppressive agent of choice. Antiplatelet agents have a limited role except in the palliation of the syndrome of erythromelalgia. Median survival is approximately 10 years. As implied above, the causes of morbidity and mortality vary with the mode of chronic therapy which has been employed, leukemia being more common after myelosuppressive therapy and thrombotic complications being more common after therapy with phlebotomy alone. Ten percent to 50% of patients move into a spent phase followed by postpolycythemic myeloid metaplasia, irrespective of previous therapy employed. Eventually, the major problems may be cytopenias and massive splenomegaly.
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PMID:Polycythemia vera. 158 7

An immunohistochemical and morphometric study was performed on routinely processed trephine biopsies of the bone marrow in 30 normal individuals and in 90 patients with various subtypes of chronic myeloproliferative disorder. Using a new monoclonal antibody (PG-M1) directed against a formalin-resistant epitope on macrophages and by employment of the Prussian blue reaction, quantitation of this cell population was feasible. Morphometric analysis revealed that the number of iron-laden macrophages represented only a fraction of the total number of histiocytic reticular cells. As could be expected, in polycythaemia rubra vera, no haemosiderin deposits were detectable, but the content of macrophages slightly exceeded that of the normal bone marrow. In chronic myeloid leukaemia 9 of 30 patients showed a significant increase in PG-M1-positive reticular cell elements. These were consistent with pseudo-Gaucher cells, sea-blue histiocytes and intermediate cell types. Primary (idiopathic) myelofibrosis-osteomyelosclerosis was characterized by a significant increase in macrophages (25 of 30 patients). Involvement of macrophages in the complex mechanisms generating bone marrow fibrosis and angiogenesis and in bone remodelling (osteosclerosis) may be responsible for this finding.
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PMID:Macrophages in normal human bone marrow and in chronic myeloproliferative disorders: an immunohistochemical and morphometric study by a new monoclonal antibody (PG-M1) on trephine biopsies. 163 47

Some routine red blood cell (RBC) measurements and indexes (count, mean volume, volume dispersion, and mean hemoglobin [HGB] concentration) can be used to differentiate iron deficiency from heterozygous beta-thalassemia. A number of formulas that incorporate two or more of these measurements have been described to amplify such differences. The H*1 hematology analyzer directly measures volume and HGB concentration of individual RBCs. We have assessed the diagnostic usefulness of conventional and new RBC measurements provided by the H*1 on a learning data set that comprised 119 patients with iron deficiency and 172 patients with beta-thalassemia trait, both untreated and uncomplicated. The most striking finding was the inverse behavior of percentages of microcytes (volume, less than 60 fL) and hypochromic RBCs (HGB concentration, less than 280 g/L) in the two conditions. In 162 of 172 patients with beta-thalassemia trait, the percentage of microcytes (mean, 33.1%; central 95th percentile range, 9.2% to 54.5%) was higher than the percentage of hypochromic RBCs (mean, 13.9%; central 95th percentile range, 1.7% to 24.7%). In 105 of 119 patients with iron deficiency, on the contrary, the percentage of hypochromic cells (mean, 34.6%; central 95th percentile range, 9.7% to 73.1%) was higher than the percentage of microcytes (mean, 12.8%; central 95th percentile range, 1.7% to 29.6%). The ratio between the percentage of microcytes and the percentage of hypochromic cells provided by the H*1 (microcytic-hypochromic ratio) was useful in differentiating the two types of microcytic anemia: with the use of a discriminant value of 0.9, the discriminant efficiency of the microcytic-hypochromic ratio was 92.4% (95% confidence interval, 88.8% to 95.2%), higher than that of the five previously described discriminant formulas and simple RBC measurements. When assessed on a test data set that comprised 149 unselected cases of microcytic anemia, a microcytic-hypochromic ratio lower than 0.9 demonstrated high sensitivity (94.0%), specificity (92.3%), and predictive value (94.0%) for the presence of iron-deficient erythropoiesis in patients with isolated iron deficiency, polycythemia vera treated by phlebotomy, and iron deficiency complicating heterozygous thalassemia. In conclusion, our results showed that iron-deficient erythropoiesis is characterized by the production of RBCs with a severely decreased HGB concentration, while microcytes of beta-thalassemia trait are generally smaller, with a more preserved HGB concentration. Such properties, as assessed by the H*1 hematology analyzer, are very useful in distinguishing these two common types of microcytic anemia.
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PMID:Automated measurement of red blood cell microcytosis and hypochromia in iron deficiency and beta-thalassemia trait. 173 38

We have previously reported that the chemiluminescence (CL) response of neutrophils (PMN) from patients with polycythemia vera (PV) was abnormally low when induced by surface receptor-dependent stimuli, fMLP and leukotriene B4, but normal when elicited by phorbol myristate acetate (PMA). This study documents that this discrepancy of the CL response to fMLP and PMA remained over a wide range of stimuli concentrations, was not due to iron-deficient PV cells and was also observed with the nitroblue tetrazolium assay. Moreover, another surface receptor-dependent agonist, platelet-activating factor, conferred a significantly lower CL response in PV PMN relative to controls. Treatment with alpha interferon or GM-CSF, to increase fMLP receptors, resulted in a similar enhancement of fMLP-induced CL in PV and controls. CL was normal when induced by a number of non-surface receptor-dependent stimuli. Release of lactoferrin in response to fMLP (and PMA) was normal (as was previously reported fMLP-induced chemotaxis and adherence). Thus, this defect is highly specific for oxidative metabolism, and localized to discrete step(s) of the stimulus-response coupling for fMLP, leukotriene B4 and PAF, but conceivably not due to impairment of the dynamic interaction of fMLP with its receptor.
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PMID:Further studies of the defective stimulus-response coupling for the oxidative burst in neutrophils in polycythemia vera. 195 81


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