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)

Chromosome 1 is known to often be involved in various malignant diseases. Its numerical and structural aberrations have been observed in chronic and acute leukemias and solid tumors as well. Recently five protooncogenes have been assigned to the long and short arms of chromosome 1. The frequent and nonspecific occurrence of chromosome 1 rearrangements in human tumors suggests that they play an important role in the pathogenesis and progression of these diseases. The frequency, types, and time of the occurrence of chromosome 1 aberrations and their relation to the stage of the disease were studied in 317 patients with various malignant diseases. In ten patients nonrandom aberrations of chromosome 1 were observed. Two patients had CML, two PRV followed by ANLL, and the remaining six patients suffered from ANLL, ALL, Burkitt lymphoma, MF, SMMoL, and IRSA, respectively. In six patients, total or partial trisomy of the long arm or of the whole chromosome 1 was present, and in three cases balanced translocations involving chromosome 1 could be found. In the cells of one patient a duplication of the centromeric heterochromatin was seen. We analyzed the breakpoints involved. Finally, the aberrations of chromosome 1 were almost always be observed at the terminal stage of the diseases.
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PMID:Abnormalities of chromosome 1 in relation to human malignant diseases. 259 63

Two patients who developed acute lymphoblastic leukaemia following polycythaemia rubra vera (PRV) are described. In both cases the diagnosis was made using cytochemistry and immunological markers. One patient's cells marked as T-cell acute lymphoblastic leukaemia (T-ALL), the other as unclassified (null) ALL. These cases support the concept that PRV is a stem cell disorder.
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PMID:Polycythaemia rubra vera transforming to acute lymphoblastic leukaemia. 347 36

N4-Palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) was administered p.o. to 199 patients with acute leukemia, myelodysplastic syndromes (MDS) and myeloproliferative disorders (MPD). Of 76 patients with AML, 11 achieved complete remission (CR) and 7 achieved partial remission (PR). Of 8 patients with ALL, 2 achieved CR and 1 achieved PR. Of 3 patients with blast crisis of MPD, 1 achieved CR. CR was reached with PL-AC at 100-900 mg/day after 5-98 (median 26) days. Of 50 patients with MDS, 2 achieved CR, 2 showed good response and 7 partial response. Response was reached with 100-400 mg/day after 13-122 (median 32) days. Improvement of polycythemia vera was observed in 6 of 13 patients, and reduction of thrombocytosis was observed in 20 of 23 patients with essential thrombocythemia and myelofibrosis. Of 18 patients with CML, 1 achieved CR. Major side effects were GI toxicities and myelosuppression. In spite of the disadvantages of the oral form of the drug, such as unpredictable absorption, PL-AC may be useful in the treatment of acute leukemia, especially that of the aged, a condition for which intensive chemotherapy is not always indicated, and MDS, which do not necessarily require admission to a hospital.
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PMID:[A phase II study of N4-palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) in patients with acute leukemia and myelodysplastic syndromes. Cooperative Study Group for PL-AC]. 361 59

From August 1979 to April 1981, 33 consecutive patients with malignant hematological diseases, entered this phase II study. Sixteen patients had NHL, eight CLL, four Myeloma, three HD, one ALL, and one Polycythaemia vera. Two patients were unevaluable because of early death. The median age was 67 years. Eight patients were not pretreated with drugs. Two CR (5+, 20+ weeks) were obtained among NHL patients, whereas five PR were observed among two NHL, one CLL, one Myeloma, and one HD patients, respectively. Toxicity was almost exclusively hematologic and occurred in ten patients, in one of them causing severe myelosuppression. Moreover, severe asthenia, attributable to VM26, was encountered in three patients, in one requiring the suspension of the treatment.
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PMID:VM26 in malignant hematological diseases. A phase II study. 695 64

In a variety of human tumors, including high grade Non-Hodgkin's lymphoma (hgNHL), a linkage between expression of CD44 variant isoforms (CD44v) and tumor progression has been described. In search of an easily accessible diagnostic parameter, expression of CD44 standard (CD44s) and CD44 variant isoforms (exons v5, v6, v7 and v10) in peripheral blood lymphocytes (PBLs) of patients with hematological malignancies was evaluated by fluorescence activated cell scanning. The analysis of 30 blood samples of healthy donors and patients with non-malignant diseases and of 183 blood samples of patients with malignant hematological disorders revealed that only in patients with malignant disorders did a measurable proportion of PBLs express CD44 variant isoforms, mostly exons v5, v6, v7 and, less frequently, exon v10. Elevated levels of CD44v expression were noted in PBLs of patients with acute and chronic myeloid leukemia (AML: 16%, CML: 25%), Hodgkin's disease (HD: 17%), multiple myeloma (MM: 22%), polycythemia vera (PV: 33%), acute lymphoid leukemia (ALL: 23%) and, most frequently, in PBLs of patients with non-Hodgkin's lymphoma (NHL:54%). CD44v expression was not restricted to the malignant phenotype, but instead was also noted in T cells, B cells and monocytes, preferentially in a subpopulation of large cells. Furthermore, expression of CD44v in PBLs was not linked to the histological grading or clinical staging. There was, however, an inverse correlation with tumor progression, whereas response to therapy was frequently accompanied by upregulation of CD44v. Thus, expression of CD44v in the PBLs of patients with NHL mainly reflected immune responsiveness. Since NHL manifests itself primarily in lymphoid organs, its progression is difficult to follow. Monitoring of CD44v in PBLs could be used as an additional and convenient parameter for surveying the course of disease.
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PMID:Expression of CD44 variant isoforms in peripheral blood leukocytes in malignant lymphoma and leukemia: inverse correlation between expression and tumor progression. 896 Jan 9

The telomerase activity of various hematologic disorders, including malignant and non-malignant ones is discussed in this paper. In total of 137 cases, each positivity of telomerase activity was MDS = 17/51, overt leukemia from MDS = 6/15, AML = 17/21, ALL = 4/6, CML-CP (chronic phase) = 0/10, CML-BC (blastic crisis) = 4/4, MPD (myeloproliferative disease)-BC = 3/3, CLL = 1/10, MM (multiple myeloma) = 0/6, aplastic anemia = 3/5, essential thrombocytosis = 0/3, and polycythemia vera = 1/3. The MPD-BC showed very high level of telomerase activity as well as CML-BC cases. From the analysis for 18 cases of AML and/or malignant lymphoma patients, significant results showed that the expression of cyclin D/E was not related to telomerase activity in these hematologic disease, as was not the case with breast cancer which was reported formerly.
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PMID:[Analysis for telomerase activity in various hematologic disorders]. 961 44

Patients with polycythemia vera (PV) are most often treated with phlebotomy-only (PHL-O) or phlebotomy plus hydroxyurea (PHL + HU). Such treatment is often unsatisfactory because of persistent susceptibility to thrombosis owing to inadequate control of abnormal erythropoiesis and thrombopoiesis. Recombinant interferon-alpha (rIFN alpha) inhibits erythroid progenitors and affects megakaryocyte function and thus may be a more effective treatment, but reports of its use have been of relatively short duration. The long-term use (median, 13 years) of rIFN alpha in 55 patients previously treated with PHL alone or with PHL + HU was studied. Data pertaining to the natural history of the disease were also examined. Patients achieved partial response of their disease by 6 months, and complete response by 1-2 years (phlebotomy-free, HCT < or =45%, platelets < or =600,000/microL); spleen size was reduced in 27 of 30 patients with prior splenomegaly. The initial dose of rIFN alpha was 1 mega unit 3 times a week (1 MU/tiw) for the majority of patients, with periodic dose increases as required and as tolerated. The maintenance dose, usually 3 MU/tiw, could be decreased after the second year of treatment in half the patients. Toxicity was acceptable. Disease-free survival was marked by no thrombohemorrhagic complications reflecting both the effect of rIFN alpha and total patient care. Evidence is presented indicating that rIFN alpha effectively reduces PHL requirements, thrombocythemia, splenomegaly, and thrombohemorrhagic events. It is an effective drug for treating PV with acceptable toxicity.
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PMID:Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha. 1680 23