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)

A total of 117 cases with hematological malignancies were treated with MCNU at doses of 70-100 mg/m2. Following are the results obtained. 1. MCNU showed a marked depression of cells in the cases with CML, polycythemia vera and thrombocythemia. The low level of cells was maintained for 2 to 7 months. 2. A good response was observed in several cases with blastic crises of CML. 3. No response was observed in two cases with acute leukemia. 4. Although a fair response was observed in several cases with malignant lymphoma or multiple myeloma, moderate bone marrow suppression was observed in a majority of the cases.
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PMID:[Phase II study with methyl-6[[[2-chloroethyl) nitrosoamino] carbonyl] amino]-6-deoxy-alpha-D-glucopyranoside (MCNU) in hematological malignancies]. 634 81

We report the detailed karyotypic analysis and clinical features of six patients with erythroleukemia (EL). Five of six patients studied displayed substantial numeric and structural chromosome abnormalities. The most common alterations in these patients were monosomy for chromosome 7 and 16. All five patients displaying chromosomal abnormalities presented with 100 percent abnormal metaphases in their bone marrow at the time of initial diagnosis. The remaining patient was studied only during remission and had a normal diploid karyotype in all marrow cells analyzed. No patient in this study had either a Ph1 -chromosome (characteristic of CML), or translocations of chromosome #8-#21 (characteristic of AML-M2). Clinically, all but one patient had a brief history; the exception having had polycythemia rubra vera for 18 years prior to the onset of EL. All patients were treated with current Southwest Oncology Group (SWOG) protocols using cytosine arabinoside and anthracycline combinations. Three of five patients entered complete remission. However, remission durations were short (approximately six months) and median survival just over one year. Cytogenetic analysis of three patients in hematologic remission revealed persistence of chromosomal alterations. It is suggested that such remissions be reclassified as partial rather than complete based upon the cytogenetic information.
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PMID:Cytogenetic and clinical assessment of six patients with erythroleukemia. 657 67

Isozymes of amino acid naphthylamidase (called B and C) with deviating electrophoretic mobilities were found in peripheral blood leukocytes of 25 out 25 untreated acute and chronic myeloid leukemias (AML and CML) and in 2 out of 2 cases of idiopathic myelofibrosis, while a normal pattern was found in 3 control groups and 5 cases of polycythemia vera. In the AML group, a correlation between electrophoretic mobility and the number of blast cells was found, and on remission, the B isozyme mobility was nearly normalized. Thus, deviating electrophoretic mobility of the B isozyme seemed to be valuable for diagnosis of myeloid leukemias, and in the AML group, the change in mobility might indicate the size of the leukemic clone.
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PMID:Discriminative value of isozymes of amino acid naphthylamidase in the diagnosis of myeloid leukemias. 693 Mar 22

Human pluripotent hemopoietic progenitors (CFU-GEMM) from mixed colonies when cultured in methylcellulose or agar in the presence of erythropoietin and PHA-LCM. The observed frequency is low and varies for different individuals between 0 and 4 mixed colonies/O5 mononuclear cells. CFU-GEMM do not adhere to glass or plastic surfaces; their density is less than 1.077 g/ml, and their sedimentation velocity profile peaks at 4.5 mm/h. They do not form rosettes with sheep red blood cells. These physical parameters can be used preparatively to enrich for CFU-GEMM to facilitate assessment of their biological properties such as cycle state analysis and measurement of self-renewal capacity. Preliminary information suggests that some CFU-GEMM are capable of self-replication. Cycle state data are available for a larger number of patients with various clinical conditions. CFU-GEMM were found to be quiescent under steady-state conditions. They proliferate actively during bone marrow regeneration and in stem cell disorders like Polycythemia rubra vera or CML. These changes in cycle state activity were not reflected in numerical alterations of CFU-GEMM. It was thus concluded that the assay may be used more meaningfully to assess biological properties of human pluripotent progenitors rather than their frequency.
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PMID:Properties of human pluripotent hemopoietic progenitors. 693 20

We have previously demonstrated the presence of a reverse transcriptase-like enzyme in retroviral particles from patients with essential thrombocythemia, polycythemia vera, and chronic myelogenous leukemia. It was subsequently shown that the human genome contains 50 copies of HERV-K. HERV-K is a human endogenous class I retroviral element that contains gag, pol, and env open reading frames. Using both reverse transcriptase-polymerase chain reaction and ribonuclease protection assays, it is demonstrated that the HERV-K pol is expressed in human blood leukocytes. The data indicates that this expression is restricted in CML white cells and is the result of gene regulation.
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PMID:Expression of human endogenous retrovirus (HERV-K) in chronic myeloid leukemia. 750 41

Morphometric analysis of sections of biopsy specimens from patients with chronic myeloproliferative disorders (CMPD) can complement the individual histological diagnosis and help to distinguish the four groups of CMPD. A total of 130 diagnostic biopsies from 29 cases of chronic myelocytic leukemia (CML.CT), 26 cases of (CML.MI), 28 of essential thrombocythemia (PTH), 26 cases of chronic megakaryocytic granulocytic myelosis (CMGM), and 21 of polycythemia vera (P. vera), and 30 from healthy control persons were evaluated morphometrically in sections of undecalcified plastic-embedded core biopsies. Clear distinctions were revealed in size of megakaryocytes, nuclear lobulation, clustering, and the nuclear size and shape of megakaryocytes. Nuclear size and cellular size were significantly less in CML (range of means of cellular size: 220-360 microns2) than in the other three Ph1-negative groups (range of means: 480-750 microns2). Nuclear lobulation was more distinct in PTH than in P. vera, and especially in CMGM. Clustering of megakaryocytes was more than twice as frequent in CMGM (8.0-10.5%) as in any of the other three groups (0.1-7.0%). Naked nuclei were more numerous in all groups of CMPD. The main topic of the study is the different size of megakaryocytes in the four main groups of CMPE, allowing a distinction between small-megakaryocytic Ph1-positive CML and large-megakaryocytic Ph1-negative forms of CMPD.
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PMID:[Morphometry of megakaryocytes for supporting the histologic diagnosis of chronic myeloproliferative diseases]. 788 12

The Ph1-negative groups of chronic myeloproliferative diseases (CMPD) are described, and histopathological criteria that distinguish them from each other are given. These are based upon observations in primary biopsies from 2,331 patients with CMPDs among a total of 34,160 patients referred between 1 January 1989 and 30 June 1994 to the Bone Marrow Registry. These cases of CMPD break down into the main groups as follows: CML 23.2%, megakaryocytic myelosis consistent with agnogenic myeloid metaplasia 22.3%, essential thrombocythemia 22.1%, and polycythemia vera 20.4%; 12.0% of cases were unclassifiable. Histological progress in each group is characterized by (1) increasing number and pleomorphy of megakaryocytes, (2) increasing fibrosis, and (3) excess of blasts. These three features can be observed in diagnostic biopsies before any therapy. Therefore, it is recommended that such alterations be reported semiquantitatively. A staging system with four stages from 0 to 3 for each of the three features is introduced. Its application allows staging for the individual patient on the basis of diagnostic biopsies.
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PMID:[Histopathology of Ph1-negative chronic myeloproliferative diseases]. 788 16

Interferon-alpha (IFN-alpha) exhibits a clear platelet reductive effect in patients with essential thrombocythemia as well as in other chronic myeloproliferative disorders with thrombocytosis. In a total of 51 patients with chronic myeloproliferative disorders with thrombocytosis we analyzed the effect of IFN-alpha in respect to platelet reduction, remission rates, induction- and maintenance dosage, long term tolerance and side effects. According to our classification CML 6, chronic mega-karyocytic granulocytic myelosis 5, essential thrombocythemia 26 and polycythemia vera 15 patients were treated. Treatment consisted of induction with 3 or 5 MU IFN-alpha daily followed by a maintenance therapy with 3 or 5 MU thrice weekly. Platelet reduction was found in all patients, CR (platelets < 450 G/l) in 78%. Within 2 months of induction therapy, CR in patients treated with 5 MU IFN daily was found in 75% compared to 52% in patients treated with 3 MU IFN daily. Dosage reduction in maintenance periode caused an increase of platelets to more than 450 G/l in 39% of patients. Out of 40 Philadelphia-negative chronic myeloproliferative disorders treated for more than 3 months in 10 patients treatment was disrupted after 5 to 18 months because of the following side effects: nausea, fatigue, vertigo, fever, headache, diarrhea, anorexia, heartburn, hairloss, myalgia, and thrombocytopenia. Due to the mutagenic effect of alkylating cytostatics and Radiophosphorus, IFN-alpha treatment represents a first line strategy for chronic myeloproliferative disorders with thrombocytosis especially in younger patients who are symptomatic and in those who suffered from episodes of bleeding or thrombosis.
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PMID:[Interferon therapy in essential thrombocythemia]. 827 65

Neutrophil granule subsets and dynamics were studied in 4 patients with polycythemia vera/myelofibrosis and 2 patients with chronic myelogenous leukemia. Alkaline phosphatase, a marker for the membrane of secretory vesicles (the most readily mobilizable pool of intracellular membranes in neutrophils) was highly elevated in the PV/MF patients and significantly reduced in the CML patients. In spite of this, the amount of secretory vesicles was normal as judged by the content of albumin, and of the membrane protein cytochrome b-245 and CD11b, both partially localized in secretory vesicles. Gelatinase granules were present in all patients. The azurophil granules were lighter than normal in both CML patients. SDS-PAGE protein profiles indicated absence of defensins from azurophil granules from 1 CML patient. In addition, a 41-42 kD doublet protein band was absent from 2 PV and 1 CML patient, and reduced in the other CML patient. No difference in mobilization of granules was observed between patient neutrophils and control neutrophils. Also, stimulation with 10(-8) mol/l N-formyl-methionyl-leucyl-phenylalanine induced normal increases in intracellular Ca2+ in patient neutrophils. These results indicate that stimulus-response coupling leading to granule exocytosis is intact in neutrophils from patients with myeloproliferative disorders.
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PMID:Mobilization of granules in neutrophils from patients with myeloproliferative disorders. 838 6

The Vav protooncogene is expressed almost exclusively in hematopoietic cells, but its role in regulating adult human hematopoietic cell development remains uncertain. To analyze Vav function in adult blood cell formation, we used antisense (AS) oligodeoxynucleotides (ODN) to disrupt its expression in normal and malignant human hematopoietic cells. Bone marrow or peripheral blood mononuclear cells (MNC) were obtained from consenting normal donors and patients with acute or chronic myelogenous leukemia (AML and CML, respectively) and polycythemia vera (PV). Adherent and T-cell-depleted (A-T-) MNC or CD34+ MNC were exposed to unmodified sense, antisense, or scrambled sequence ODN corresponding to codons 2-7 of Vav's mRNA sequence. Cells were then assayed for Vav mRNA expression by reverse transcription-polymerase chain reaction and Vav protein expression by Western binding. After showing that Vav-targeted AS ODN could specifically diminish Vav mRNA and protein expression, we assessed the ability of Vav-deficient cells to form myeloid and erythroid colonies in methyl-cellulose cultures. When normal CD34+ MNC were exposed to Vav AS ODN, no effect on colony-forming unit-granulocyte-macrophage (CFU-GM) or CFU-megakaryocyte colony formation was observed. In contrast erythroid colony growth was inhibited by a mean +/- SD of 62% +/- 16%. In patients with hematopoietic malignancies. Vav-targeted AS ODN inhibited CFU-GM colony formation in a sequence-specific and dose-dependent manner in 1 of 3 AML, 13 of 17 CML, and 2 of 2 PV patients. At the highest concentration used, the Vav AS ODN inhibited CFU-GM colony formation from 66% to 81% when compared with control cell colony growth. Burst-forming unit-erythroid (BFU-E) colony-formation was also assessed in 7 PV patients. The Vav-targeted AS ODN inhibited BFU-E colony formation in all by a mean +/- SD of 81% +/- 4%. These findings suggest that Vav function may not be easily complemented in a significant subset of normal adult erythroid progenitor cells and may also be necessary for myeloid progenitor cell growth in a variety of hematopoietic malignancies.
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PMID:A functional analysis of protooncogene Vav's role in adult human hematopoiesis. 860 21


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