1/62. Acute lymphoid leukemia following polycythemia vera.The tendency to evolve into acute leukemia is a well-known characteristic of polycythemia vera (PV), which is shared with the remaining chronic myeloproliferative disorders and increases after the administration of cytotoxic agents. Acute transformation is usually of myeloid phenotype, whereas acute lymphoid leukemia (ALL) following PV is seldom observed. A 63-year-old woman is described who developed ALL at 6 years from the initial diagnosis of PV, for which she had received radioactive phosphorus and hydroxyurea. The ALL was of B-cell type, corresponding to the L-3 subtype of the FAB classification. Despite the administration of combination chemotherapy the patient died shortly after the diagnosis of acute leukemia. The present case adds to seven previously described patients with the above association, all of whom had received cytotoxic therapy for PV. Median interval from PV to ALL diagnosis was 10 years, and there was a predominance of the B-cell phenotype. The prognosis was poor since all but one of the patients had a short survival after ALL diagnosis. The possible etiological and pathogenetic link between PV and the subsequent ALL is discussed.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/62. Polycythemia rubra vera progressing to Ph1-positive chronic myelogenous leukemia.Polycythemia rubra vera and chronic myelogenous leukemia are both myeloproliferative disorders and, as such, share certain clinical features. Although some myeloproliferative disorders may transform into others, conversion of polycythemia rubra vera to chronic myelogenous leukemia has been denied. We report here an elderly man with polycythemia rubra vera and a normal leukocyte alkaline phosphatase who developed chronic myelogenous leukemia with a low leukocyte alkaline phosphatase and a marrow karyotype of 45, X, Ph1-positive. In addition, we have collected evidence of two similar cases and thus conclude that, although uncommon, polycythemia rubra vera may on occasion progress to chronic myelogenous leukemia.- - - - - - - - - - ranking = 1.1428571428571keywords = leukemia (Clic here for more details about this article) |
3/62. Observations on polycythemia vera turning into acute or chronic granulocytic leukemia during treatment with radioactive phosphorus 32P.In a group of 172 cases of polycythemia vera treated with radioactive phosphorus acute granulocytic leukemia developed in 3 and chronic granulocytic leukemia in 6 cases. The author believes that development of acute granulocytic leukemia during this treatment may be considered with some probability as a result of the leukemia-inducing action of ionizing radiation. Transition of polycythemia vera into chronic granulocytic leukemia seems to be a natural outcome of this complex myeloproliferative syndrome in patients with survival prolonged by treatment with 32P.- - - - - - - - - - ranking = 1.2857142857143keywords = leukemia (Clic here for more details about this article) |
4/62. Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.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.- - - - - - - - - - ranking = 0.71428571428571keywords = leukemia (Clic here for more details about this article) |
5/62. Leukemic transformation of polycythemia vera after treatment with hydroxyurea with abnormalities of chromosome 17.The leukemogenic risk attributed to therapy of polycythemia vera with radiophosphorus and alkylating drugs has led, over the last 20 years, to the increased use of myelosupressive nonmutagenic drugs, especially hydroxyurea. But there exist reports, which showed the development of polycythemia vera into acute leukemia not only in patients treated with alkylating agents and radiophosphorus but also with single hydroxyurea. In this article we present two cases of polycythemia vera, in which the development to acute myeloblastic leukemia occurred after long-term treatment with hydroxyurea. Significant is the fact, that in both presented cases cytogenetic and FISH analysis showed abnormalities of chromosome 17, in the one of case fullfilled criteria for "17p-syndrome". Due to the possibility of leukemogenic potential in the time of hydroxyurea treatment, it is necessary to be careful especially in young patients. The dynamic follow up of cytogenetic analysis is necessary, especially, in those, where long-term hydroxyurea therapy is supposed.- - - - - - - - - - ranking = 0.28571428571429keywords = leukemia (Clic here for more details about this article) |
6/62. Abnormalities of chromosome 1 in myeloproliferative disorders.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.- - - - - - - - - - ranking = 0.42857142857143keywords = leukemia (Clic here for more details about this article) |
7/62. Simultaneous occurrence of polycythemia vera and waldenstrom macroglobulinemia: a case report and review of the literature.polycythemia vera and lymphoproliferative disorders can occur simultaneously. lymphoproliferative disorders reported to occur simultaneously with polycythemia vera include chronic lymphocytic leukemia (CLL), non-Hodgkin's lymphoma (NHL), and acute lymphoblastic leukemia (ALL). Different theories have attempted to explain this occurrence. Some implicate a common cell of origin to the two disorders, others include chance alone, yet others implicate an underlying humoral or immune mechanism caused by the first disorder leading to a second clonal expansion. We report the first case of polycythemia vera with a simultaneous diagnosis of waldenstrom macroglobulinemia, and we review the literature trying to explain this unusual occurrence.- - - - - - - - - - ranking = 0.28571428571429keywords = leukemia (Clic here for more details about this article) |
8/62. A novel t(16;20)(q22;p13) in polycythemia vera.We report a patient with polycythemia vera whose bone marrow cells carried a novel t(16;20)(q22;p13) as detected by karyotype analysis using G- and Q-banding techniques. The reciprocal translocation was confirmed by fluorescence in situ hybridization (FISH) using dna libraries of chromosomes 16 and 20. To our knowledge, t(16;20)(q22;p13) has not been reported previously. The core binding factor beta (CBFbeta) gene located on 16q22 is known to be frequently involved in acute myelocytic leukemia. On the other hand, the 20p13 locus contains a gene encoding protein kinase CK2alpha, which is closely related to cell proliferation and cell cycle regulation. The t(16;20)(q22;p13) may be one of the cytogenetic aberrations in myeloproliferative disorders, and therefore, our observation warrants further studies on a possible involvement of the genes resulting from this translocation.- - - - - - - - - - ranking = 0.14285714285714keywords = leukemia (Clic here for more details about this article) |
9/62. Megakaryoblastic transformation of polycythemia vera with hypercalcemia.polycythemia vera (PV) is known to occasionally transform into acute leukemia. Administration of alkylating agents seems to be associated with an increased risk of leukemic transformation of PV. hypercalcemia is a serious complication of malignancies, but it is uncommon in acute leukemia. In the majority of malignancies with hypercalcemia, elevated parathyroid hormone-related protein (PTHrP) is thought to be the main cause of hypercalcemia. We report a rare case of megakaryoblastic transformation of PV with hypercalcemia. A 62-year-old man was diagnosed as having PV in 1983, and he had received ranimustine and busulfan. He developed acute megakaryoblastic leukemia 17 years after the initial diagnosis of PV. serum calcium was elevated, the serum level of intact parathyroid hormone (PTH) was suppressed, and the level of intact PTHrP was slightly elevated. He had no lytic bone lesions; however, uncoupling of bone turnover due to an increase in bone resorption and a decrease in bone formation was detected by using biochemical markers. Since the level of PTHrP was slightly elevated from the normal value, we speculated that PTHrP produced in the local field by leukemic cells might have been more abundant than circulating PTHrP. Pamidronate and adrenocortical hormone were effective in reducing the serum calcium level. However, the patient died shortly after the start of induction chemotherapy. The prognosis of cases of PV that has transformed into acute leukemia is generally poor because the majority of such cases are refractory to chemotherapy.- - - - - - - - - - ranking = 0.57142857142857keywords = leukemia (Clic here for more details about this article) |
10/62. Translocation (X;20)(q13.1;q13.3) as a primary chromosomal finding in two patients with myelocytic disorders.Reports of x chromosome translocations, as primary chromosomal changes associated with hematologic disorders, remain relatively uncommon. Herein, we report the detection, by conventional cytogenetic methods, of a cytogenetically identical t(X;20) in two different patients with hematologic disorders (probable myelodysplasia and polycythemia vera/acute myelocytic leukemia). In both cases, this translocation appeared as the primary clonal chromosome abnormality, with breakpoints occurring in the long arms of both the x chromosome and chromosome 20 (Xq13.1 and 20q13.3, respectively). Further characterization and comparison of the translocation chromosome products of these two cases by use of fluorescence in situ hybridization techniques is also described. Similar previously reported cytogenetically cases and the potential that this specific rearrangement may represent a nonrandom chromosomal finding are discussed.- - - - - - - - - - ranking = 0.14285714285714keywords = leukemia (Clic here for more details about this article) |
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