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)

Cytogenetic studies were done on 18 patients with myelofibrosis or the closely related syndrome, undifferentiated myeloproliferative disorder (MPD). Clones of cells with chromosome abnormalities were demonstrated in the blood of eight individuals, including two with a history of radiation therapy and two with "acute myelofibrosis". Trisomy 8 was present in the latter two patients, but otherwise, there was no consistent cytogenetic pattern or correlation with specific hematologic findings. Sixteen of these patients have been followed for more than 1 year or until death; none has progressed to leukemia. The results indicate that chromosome abnormalities are relatively common in this disorder, but as with polycythemia vera, and unlike some other "preleukemic" states, the aberrant clones in myelofibrosis do not appear to indicate that clinical leukemia is imminent.
Cancer 1976 Nov
PMID:Chromosome studies in "preleukemia". III. Myelofibrosis. 99 Nov 2

Ionizing radiation used for diagnosis or therapy has been associated with an increased incidence of malignancies of blood-forming organs. The increased incidence of hematopoietic malignancies following exposure to ionizing radiation obtained in the course of occupation, diagnosis and therapy of disease, or as a weapon of war is documented. The natural occurrence and the induced progression to acute leukemia of polycythemia rubra vera, Hodgkin's disease, multiple myeloma, Di Guglielmo's disease, and reticuloendothelial malignancies are discussed. The status of transplantation and immunodeficiency states and their relationship to acute leukemia is reviewed. Finally, drugs, toxins, and the use of cytotoxic radiomimetic agents for nonmalignant purposes are shown to lead to the development of acute leukemia. Background information relevant to the proper use of future diagnostic and therapeutic modalities is provided.
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PMID:Malignancies in blood-forming organs following diagnostic and therapeutic procedures: a review. 106 32

Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.
Cancer 1976 Dec
PMID:Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera. 106 2

Chromosomal findings are reported in three patients with acute myelomonocytic leukemia and in one with reticulosarcoma leukemia who had been treated for multiple myeloma with melphalan and X-ray. All four patients had striking chromosomal anomalies. An iatrogenic causation of aneuploidy is suggested. This is supported by chromosomal findings in patients with acute leukemia following polycythemia vera and Hodgkin's disease; practically all of the leukemias have been aneuploid. A comparison is made of such "secondary" acute leukemias with "primary" acute leukemias that are aneuploid in only 40% of the cases. Chromosomal changes are not considered to be the initial event in leukemogenesis.
Cancer Res 1975 Oct
PMID:Chromosome studies in acute leukemias developing in patients with multiple myeloma. 109 66

Three patients with myeloproliferative disorders showed a similar chromosome abnormality, accompanied by other abnormalities that were different in each case. Marrow cells from all three patients were trisomic either for the entire chromosome 1 or for its long arm. Patient 1 had a brief period of anemia and thrombocytopenia which preceded a terminal acute leukemia; Patient 2 had polycythemia vera (P.V.) that terminated in acute leukemia; and Patient 3 has P.V. The detection of an abnormal karyotype in Patients 1 and 2 was an important factor in establishing the diagnosis of acute leukemia. Preliminary evidence supports the suggestion that some chromosomal changes are nonrandom in myeloproliferative diseases. Nonrandom abnormalities involving the same chromosome have been observed in several human neoplastic disorders.
Cancer 1975 Nov
PMID:Abnormalities of chromosome 1 in myeloproliferative disorders. 119 63

The chromosome constitution of bone marrow cells was determined in 13 patients with polycythemia vera (PV). In 5 of these patients definite karyotypic abnormalities were found: 3 with 46,XY,Fq-; 1 with 46,XY,11q-, 13q-; and 1 with missing Y. The latter cytogenetic finding is thought not to be related to the PV. The 4 patients with partial deletions of chromosomes had been treated with 32P, and 3 of them with chemotherapy also. Karyotypes from 2 of these patients, 1 with 46,XY,20q-, and another with 46,XY,11q-,13q- were examined with G and/or Q banding. From the results of the banding analysis, it appears that the abnormal chromosomes were due to simple deletions at specific sites on their arms, particularly in the cases with the F abnormality (20q11). The breaks occurred in the regions C11q and D12q. The portions missing from the original chromosomes could not be found on any chromosome. Most patients with PV do not develop chromosomal abnormalities through the course of their disease; when such abnormalities appear (20q- in particular), they seem to result exclusively from radiation (and possibly chemo-) therapy. Thus, cytogenetic changes do not appear to play a crucial role in the genesis of PV.
Cancer 1975 Jul
PMID:Chromosomes and causation of human cancer and leukemia. XII. Banding analysis of abnormal chromosomes in polycythemia vera. 120 47

There is a long history recording the association of x radiation and the subsequent development of malignant tumors. For systematically administered isotopes this came into prominence when Martland discovered the association between cancer, particularly of the bone, and ingestion of radioactive isotopes by radium dial painters. This association was amplified by the development of cancer in patients given thorotrast as a contrast medium for diagnostic radiologic examination. Acute leukemia was reported 30 years ago in patients with polycythemia vera treated with 32P. Acute leukemia also occurs in patients with polycythemia vera treated only with phlebotomy or drugs. A controlled study is now underway to provide a more definite answer to question what is the incidence of acute leukemia in patients with polycythemia vera treated by phlebotomy alone, chlorambucil, or 32P. 131I for the treatment of hyperthyroidism probably does not induce cancer, but in the doses used for thyroid cancer there was an increased incidence of neoplasms (12/200 in one study). This was higher than the expected incidence of neoplasms. The doses of radioactive isotopes used currently for diagnostic purposes have not induced cancer, but it is difficult and probably impossible to verify this with absolute certainty.
Cancer 1976 Feb
PMID:The association between systemically administered radioisotopes and subsequent malignant disease. 125 26

Recent studies have generated data demonstrating significant clinical activity of alpha-interferon therapy in each of six hematological malignancies, chronic myeloid leukaemia, essential thrombocythemia, polycythemia rubra vera, non-Hodgkin's lymphomas, multiple myelomatosis and hairy cell leukaemia.
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PMID:alpha-Interferon in hematological malignancies. 136 59

Serum erythropoietin (Epo) titers in patients with various hematological malignancies and related diseases were determined by radioimmunoassay. Serum Epo titer was inversely correlated with hemoglobin concentration in iron deficiency anemia, aplastic anemia, myelodysplastic syndromes (MDS), acute leukemia, malignant lymphoma, multiple myeloma and myelofibrosis, but there was no correlation between serum Epo titer and hemoglobin concentration in chronic myelogenous leukemia or polycythemias. Serum Epo titers in aplastic anemia were much higher than those in iron deficiency anemia. Serum Epo titers in MDS, malignant lymphoma and multiple myeloma differed considerably among patients. Serum Epo titers in untreated polycythemia vera were significantly lower than in treated polycythemia vera or secondary polycythemia.
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PMID:Serum erythropoietin titers in hematological malignancies and related diseases. 146 Mar 22

Between 1980 and 1991, 96 patients with documented polycythemia vera were treated by hydroxyurea or pipobroman, according to a protocol including randomization, and maintenance therapy. Complete remission was induced in all cases. Two cases treated with hydroxy-urea had a very severe granulothrombocytopenia during the initial phase. Maintenance was generally satisfactory on pipobroman, but the platelet count often remained high (400 to 900.10(9)/l) on low-dosage hydroxy-urea, with a risk of vascular events. Progressive resistance to these drugs was observed in 5 cases. Digestive and cutaneous troubles were more frequent on pipobroman maintenance, sometimes enough to legitimate a therapeutic change. It may be concluded that such a treatment is less easy to use and to follow than is currently accepted. In the present series (397/years/patients follow-up, median 5-3 years), only one leukemia and one cancer were observed, which however only demonstrates the absence of any carcinogenic risk at short- but not at long-term.
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PMID:[Treatment of polycythemia vera with hydroxyurea or pipobroman. Efficacy and toxicity analysed from a protocol of 96 patients under 65 years of age. Le Groupe d'Etude des Polyglobulies]. 148 20


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