polycythemia vera

Summary

Summary: A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.

Top Publications

  1. Hasselbalch H. A role of NF-E2 in chronic inflammation and clonal evolution in essential thrombocythemia, polycythemia vera and myelofibrosis?. Leuk Res. 2014;38:263-6 pubmed publisher
    ..Accordingly, this novel murine model may also have delivered the proof of concept of chronic inflammation as a trigger and driver of clonal evolution in MPNs. ..
  2. Berkofsky Fessler W, Buzzai M, Kim M, Fruchtman S, Najfeld V, Min D, et al. Transcriptional profiling of polycythemia vera identifies gene expression patterns both dependent and independent from the action of JAK2V617F. Clin Cancer Res. 2010;16:4339-52 pubmed publisher
    To understand the changes in gene expression in polycythemia vera (PV) progenitor cells and their relationship to JAK2V617F...
  3. Hussein K, Bock O, Theophile K, von Neuhoff N, Buhr T, Schlue J, et al. JAK2(V617F) allele burden discriminates essential thrombocythemia from a subset of prefibrotic-stage primary myelofibrosis. Exp Hematol. 2009;37:1186-1193.e7 pubmed publisher
    ..Thus, for Ph(-) MPN in which ET and prefibrotic PMF represent the most probable diagnoses, a JAK2(V617F) allele burden >50% favors a diagnosis of prefibrotic PMF. ..
  4. Boissinot M, Cleyrat C, Vilaine M, Jacques Y, Corre I, Hermouet S. Anti-inflammatory cytokines hepatocyte growth factor and interleukin-11 are over-expressed in Polycythemia vera and contribute to the growth of clonal erythroblasts independently of JAK2V617F. Oncogene. 2011;30:990-1001 pubmed publisher
    ..activating mutation of janus kinase 2 (JAK2), a kinase essential for cytokine signalling, characterizes Polycythemia vera (PV), one of the myeloproliferative neoplasms (MPN)...
  5. Rumi E, Harutyunyan A, Elena C, Pietra D, Klampfl T, Bagienski K, et al. Identification of genomic aberrations associated with disease transformation by means of high-resolution SNP array analysis in patients with myeloproliferative neoplasm. Am J Hematol. 2011;86:974-9 pubmed publisher
    Myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF)...
  6. Berlin N, Berlin N. Polycythemia vera. Hematol Oncol Clin North Am. 2003;17:1191-210 pubmed
    The differential diagnosis of an elevated hematocrit and the criteria for the diagnosis of polycythemia vera present little or no problem; however, there is not a consensus on therapy...
  7. Ugo V, Tondeur S, Menot M, Bonnin N, Le Gac G, Tonetti C, et al. Interlaboratory development and validation of a HRM method applied to the detection of JAK2 exon 12 mutations in polycythemia vera patients. PLoS ONE. 2010;5:e8893 pubmed publisher
    ..that the V617F mutation in the exon 14 of the JAK2 gene is present in about 90% of patients with Polycythemia Vera (PV), the detection of this mutation has become a key tool for the diagnosis of these patients...
  8. Tefferi A. Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management. Am J Hematol. 2012;87:285-93 pubmed publisher
    b>Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively...
  9. Hasselbalch H. Perspectives on chronic inflammation in essential thrombocythemia, polycythemia vera, and myelofibrosis: is chronic inflammation a trigger and driver of clonal evolution and development of accelerated atherosclerosis and second cancer?. Blood. 2012;119:3219-25 pubmed publisher
    ..with the chronic Philadelphia-negative myeloproliferative neoplasms (MPNs), essential thrombocythemia, polycythemia vera, and primary myelofibrosis are mainly caused by cardiovascular diseases, thrombohemorrhagic complications, ..

More Information

Publications74

  1. Perna F, Gurvich N, Hoya Arias R, Abdel Wahab O, Levine R, Asai T, et al. Depletion of L3MBTL1 promotes the erythroid differentiation of human hematopoietic progenitor cells: possible role in 20q- polycythemia vera. Blood. 2010;116:2812-21 pubmed publisher
    ..on chromosome 20q12, within the common deleted region identified in patients with 20q deletion-associated polycythemia vera, myelodysplastic syndrome, and acute myeloid leukemia...
  2. Chu C, Zhang L, Dhayalan A, Agagnina B, Magli A, Fraher G, et al. Torque teno virus 10 isolated by genome amplification techniques from a patient with concomitant chronic lymphocytic leukemia and polycythemia vera. Mol Med. 2011;17:1338-48 pubmed publisher
    ..A patient with concomitant chronic lymphocytic leukemia (CLL) and polycythemia vera (PV) requiring therapeutic phlebotomy donated a large amount of phlebotomized blood to test this possibility...
  3. Laubach J, Fu P, Jiang X, Salter K, Potti A, Arcasoy M. Polycythemia vera erythroid precursors exhibit increased proliferation and apoptosis resistance associated with abnormal RAS and PI3K pathway activation. Exp Hematol. 2009;37:1411-22 pubmed publisher
    b>Polycythemia vera (PV) is characterized by erythrocytosis associated with the presence of the activating JAK2(V617F) mutation in a variable proportion of hematopoietic cells...
  4. Stein B, Williams D, O Keefe C, Rogers O, Ingersoll R, Spivak J, et al. Disruption of the ASXL1 gene is frequent in primary, post-essential thrombocytosis and post-polycythemia vera myelofibrosis, but not essential thrombocytosis or polycythemia vera: analysis of molecular genetics and clinical phenotypes. Haematologica. 2011;96:1462-9 pubmed publisher
    The myeloproliferative neoplasms, essential thrombocytosis, polycythemia vera and primary myelofibrosis, share the same acquired genetic lesion, but the concept of JAK2 V617F serving as the sole lesion responsible for these neoplasms is ..
  5. Kubovcakova L, Lundberg P, Grisouard J, Hao Shen H, Romanet V, Andraos R, et al. Differential effects of hydroxyurea and INC424 on mutant allele burden and myeloproliferative phenotype in a JAK2-V617F polycythemia vera mouse model. Blood. 2013;121:1188-99 pubmed publisher
    ..Deleting the conditional mouse Jak2-knockout alleles increased erythropoiesis and accentuated the polycythemia vera phenotype, but did not alter platelet or granulocyte levels...
  6. Treliński J, Wierzbowska A, Krawczynska A, Sakowicz A, Pietrucha T, Smolewski P, et al. Circulating endothelial cells in essential thrombocythemia and polycythemia vera: correlation with JAK2-V617F mutational status, angiogenic factors and coagulation activation markers. Int J Hematol. 2010;91:792-8 pubmed publisher
    ..an important role in the biology of hematological malignancies, including essential thrombocythemia (ET) and polycythemia vera (PV)...
  7. Bruchova H, Yoon D, Agarwal A, Swierczek S, Prchal J. Erythropoiesis in polycythemia vera is hyper-proliferative and has accelerated maturation. Blood Cells Mol Dis. 2009;43:81-7 pubmed publisher
    b>Polycythemia vera (PV) is an acquired myeloproliferative clonal disorder, characterized by augmented erythropoiesis...
  8. Silver R, Vandris K, Wang Y, Adriano F, Jones A, Christos P, et al. JAK2(V617F) allele burden in polycythemia vera correlates with grade of myelofibrosis, but is not substantially affected by therapy. Leuk Res. 2011;35:177-82 pubmed publisher
    In a series of 105 patients with polycythemia vera, we retrospectively determined whether the JAK2(V617F) mutation correlated with severity of disease phenotype...
  9. Lu M, Wang X, Li Y, Tripodi J, Mosoyan G, Mascarenhas J, et al. Combination treatment in vitro with Nutlin, a small-molecule antagonist of MDM2, and pegylated interferon-? 2a specifically targets JAK2V617F-positive polycythemia vera cells. Blood. 2012;120:3098-105 pubmed publisher
    Interferon (IFN-?) is effective therapy for polycythemia vera (PV) patients, but it is frequently interrupted because of adverse events...
  10. Marchioli R, Finazzi G, Specchia G, Cacciola R, Cavazzina R, Cilloni D, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013;368:22-33 pubmed publisher
    Current treatment recommendations for patients with polycythemia vera call for maintaining a hematocrit of less than 45%, but this therapeutic strategy has not been tested in a randomized clinical trial.
  11. Abdulkarim K, Ridell B, Johansson P, Kutti J, Safai Kutti S, Andréasson B. The impact of peripheral blood values and bone marrow findings on prognosis for patients with essential thrombocythemia and polycythemia vera. Eur J Haematol. 2011;86:148-55 pubmed publisher
    ..Ph-) chronic myeloproliferative neoplasms include the three well-known clinical entities polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF)...
  12. Quintas Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S, et al. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol. 2009;27:5418-24 pubmed publisher
    We conducted a phase II study of pegylated interferon alfa-2a (PEG-IFN-alpha-2a) in patients with essential thrombocythemia (ET) and polycythemia vera (PV).
  13. Pardanani A, Lasho T, Smith G, Burns C, Fantino E, Tefferi A. CYT387, a selective JAK1/JAK2 inhibitor: in vitro assessment of kinase selectivity and preclinical studies using cell lines and primary cells from polycythemia vera patients. Leukemia. 2009;23:1441-5 pubmed publisher
    ..CYT387 selectively suppressed the in vitro growth of erythroid colonies harboring JAK2V617F from polycythemia vera (PV) patients, an effect that was attenuated by exogenous erythropoietin...
  14. Slezak S, Jin P, Caruccio L, Ren J, Bennett M, Zia N, et al. Gene and microRNA analysis of neutrophils from patients with polycythemia vera and essential thrombocytosis: down-regulation of micro RNA-1 and -133a. J Transl Med. 2009;7:39 pubmed publisher
    ..event in myeloprofilerative disorders (MPDs) we compared molecular changes in neutrophils from patients with polycythemia vera (PV) and essential thrombocythosis (ET), to neutrophils stimulated by G-CSF administration and to normal ..
  15. Harrison C. Rethinking disease definitions and therapeutic strategies in essential thrombocythemia and polycythemia vera. Hematology Am Soc Hematol Educ Program. 2010;2010:129-34 pubmed publisher
    ..This article, focusing mainly upon essential thrombocythemia and polycythemia vera, reviews four major themes: the impact upon classification of these disorders considering a radical review ..
  16. Alvarez Larran A, Pereira A, Cervantes F, Arellano Rodrigo E, Hernández Boluda J, Ferrer Marin F, et al. Assessment and prognostic value of the European LeukemiaNet criteria for clinicohematologic response, resistance, and intolerance to hydroxyurea in polycythemia vera. Blood. 2012;119:1363-9 pubmed publisher
    Criteria of response and definition of resistance and intolerance to hydroxyurea (HU) in polycythemia vera (PV) were proposed by the European LeukemiaNet (ELN). Such criteria were evaluated in 261 PV patients (median follow-up, 7...
  17. Silver R, Chow W, Orazi A, Arles S, Goldsmith S. Evaluation of WHO criteria for diagnosis of polycythemia vera: a prospective analysis. Blood. 2013;122:1881-6 pubmed publisher
    We prospectively evaluated the accuracy of the 2007 World Health Organization (WHO) criteria for diagnosing polycythemia vera (PV), especially in "early-stage" patients...
  18. Maugeri N, Malato S, Femia E, Pugliano M, Campana L, Lunghi F, et al. Clearance of circulating activated platelets in polycythemia vera and essential thrombocythemia. Blood. 2011;118:3359-66 pubmed publisher
    Essential thrombocythemia (ET) and polycythemia vera (PV) are characterized by persistent platelet activation. The mechanisms involved in their clearance are poorly characterized...
  19. Akada H, Yan D, Zou H, Fiering S, Hutchison R, Mohi M. Conditional expression of heterozygous or homozygous Jak2V617F from its endogenous promoter induces a polycythemia vera-like disease. Blood. 2010;115:3589-97 pubmed publisher
    A somatic point mutation (V617F) in the JAK2 tyrosine kinase was found in a majority of patients with polycythemia vera (PV), essential thrombocythemia, and primary myelofibrosis...
  20. Tefferi A, Lasho T, Abdel Wahab O, Guglielmelli P, Patel J, Caramazza D, et al. IDH1 and IDH2 mutation studies in 1473 patients with chronic-, fibrotic- or blast-phase essential thrombocythemia, polycythemia vera or myelofibrosis. Leukemia. 2010;24:1302-9 pubmed publisher
    ..were screened for isocitrate dehydrogenase 1 (IDH1)/IDH2 mutations: 594 essential thrombocythemia (ET), 421 polycythemia vera (PV), 312 primary myelofibrosis (PMF), 95 post-PV/ET MF and 51 blast-phase MPN...
  21. Spivak J. Narrative review: Thrombocytosis, polycythemia vera, and JAK2 mutations: The phenotypic mimicry of chronic myeloproliferation. Ann Intern Med. 2010;152:300-6 pubmed publisher
    The myeloproliferative disorders polycythemia vera, essential thrombocytosis, and primary myelofibrosis are clonal disorders arising in a pluripotent hematopoietic stem cell, causing an unregulated increase in the number of erythrocytes, ..
  22. Ma Y, Zhao S, Zhu J, Bettano K, Qu X, Marshall C, et al. Real-time bioluminescence imaging of polycythemia vera development in mice. Biochim Biophys Acta. 2009;1792:1073-9 pubmed publisher
    b>Polycythemia vera (PV) is a myeloproliferative disorder involving hematopoietic stem cells. A recurrent somatic missense mutation in JAK2 (JAK2V617F) is thought to play a causal role in PV...
  23. Skov V, Riley C, Thomassen M, Larsen T, Jensen M, Bjerrum O, et al. Whole blood transcriptional profiling reveals significant down-regulation of human leukocyte antigen class I and II genes in essential thrombocythemia, polycythemia vera and myelofibrosis. Leuk Lymphoma. 2013;54:2269-73 pubmed publisher
  24. Schnittger S, Bacher U, Haferlach C, Geer T, Muller P, Mittermuller J, et al. Detection of JAK2 exon 12 mutations in 15 patients with JAK2V617F negative polycythemia vera. Haematologica. 2009;94:414-8 pubmed publisher
    To further characterize JAK2 exon 12 mutations, we performed molecular screening in 409 patients with polycythemia vera or unclear erythrocytosis with unmutated JAK2V617. The frequency of JAK2exon12 mutations was 10/63 (15...
  25. Yeh Y, Chen Y, Cheng H, Su W, Chow N, Chen T, et al. High percentage of JAK2 exon 12 mutation in Asian patients with polycythemia vera. Am J Clin Pathol. 2010;134:266-70 pubmed publisher
    We examined the occurrence of JAK2(V617F) and JAK2 exon 12 mutations in a clinical cohort of polycythemia vera (PV) in Taiwan...
  26. Vannucchi A. Insights into the pathogenesis and management of thrombosis in polycythemia vera and essential thrombocythemia. Intern Emerg Med. 2010;5:177-84 pubmed publisher
    The classic myeloproliferative neoplasms (MPNs) include polycythemia vera and essential thrombocythemia; their molecular basis has been described only recently with the demonstration of recurrent mutations in JAK2 or MPL...
  27. Skov V, Thomassen M, Riley C, Jensen M, Bjerrum O, Kruse T, et al. Gene expression profiling with principal component analysis depicts the biological continuum from essential thrombocythemia over polycythemia vera to myelofibrosis. Exp Hematol. 2012;40:771-780.e19 pubmed publisher
    The recent discovery of the Janus activating kinase 2 V617F mutation in most patients with polycythemia vera (PV) and half of those with essential thrombocythemia (ET) and primary myelofibrosis (PMF) has favored the hypothesis of a ..
  28. Wu Z, Yuan H, Zhang X, Liu W, Xu J, Zhang W, et al. Development and inter-laboratory validation of unlabeled probe melting curve analysis for detection of JAK2 V617F mutation in polycythemia vera. PLoS ONE. 2011;6:e26534 pubmed publisher
  29. Risum M, Madelung A, Bondo H, Bzorek M, Kristensen M, Stamp I, et al. The JAK2V617F allele burden and STAT3- and STAT5 phosphorylation in myeloproliferative neoplasms: early prefibrotic myelofibrosis compared with essential thrombocythemia, polycythemia vera and myelofibrosis. APMIS. 2011;119:498-504 pubmed publisher
    ..clinically and histologically mimic essential thrombocythemia (ET), but is important to distinguish from ET, polycythemia vera (PV) and primary myelofibrosis (PMF) due to its different prognosis and clinical evolution...
  30. Hasselbalch H. Chronic inflammation as a promotor of mutagenesis in essential thrombocythemia, polycythemia vera and myelofibrosis. A human inflammation model for cancer development?. Leuk Res. 2013;37:214-20 pubmed publisher
  31. Barrio S, Gallardo M, Arenas A, Ayala R, Rapado I, Rueda D, et al. Inhibition of related JAK/STAT pathways with molecular targeted drugs shows strong synergy with ruxolitinib in chronic myeloproliferative neoplasm. Br J Haematol. 2013;161:667-76 pubmed publisher
    ..Furthermore, the combination of ruxolitinib with inhibitors that target these pathways has a strong synergistic effect, which may be due to decreased activation of the common effector, STAT5...
  32. Mullally A, Lane S, Ball B, Megerdichian C, Okabe R, Al Shahrour F, et al. Physiological Jak2V617F expression causes a lethal myeloproliferative neoplasm with differential effects on hematopoietic stem and progenitor cells. Cancer Cell. 2010;17:584-96 pubmed publisher
    We report a Jak2V617F knockin mouse myeloproliferative neoplasm (MPN) model resembling human polycythemia vera (PV)...
  33. Thiele J, Kvasnicka H. The 2008 WHO diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis. Curr Hematol Malig Rep. 2009;4:33-40 pubmed publisher
    Several international working groups cooperated to propose new diagnostic guidelines for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) to the steering committee of the World Health Organization...
  34. Mullally A, Poveromo L, Schneider R, Al Shahrour F, Lane S, Ebert B. Distinct roles for long-term hematopoietic stem cells and erythroid precursor cells in a murine model of Jak2V617F-mediated polycythemia vera. Blood. 2012;120:166-72 pubmed publisher
    In the current model of the pathogenesis of polycythemia vera (PV), the JAK2V617F mutation arises in hematopoietic stem cells (HSCs) that maintain the disease, while erythroid precursor populations expand, resulting in excessive red ..
  35. Olcaydu D, Harutyunyan A, Jäger R, Berg T, Gisslinger B, Pabinger I, et al. A common JAK2 haplotype confers susceptibility to myeloproliferative neoplasms. Nat Genet. 2009;41:450-4 pubmed publisher
    ..Thus, disease susceptibility loci may harbor somatic mutations that have a role in disease pathogenesis...
  36. Barosi G, Birgegard G, Finazzi G, Griesshammer M, Harrison C, Hasselbalch H, et al. Response criteria for essential thrombocythemia and polycythemia vera: result of a European LeukemiaNet consensus conference. Blood. 2009;113:4829-33 pubmed publisher
    European experts were convened to develop a definition of response to treatment in polycythemia vera (PV) and essential thrombocythemia (ET). Clinicohematologic (CH), molecular, and histologic response categories were selected...
  37. Kvasnicka H, Thiele J. Prodromal myeloproliferative neoplasms: the 2008 WHO classification. Am J Hematol. 2010;85:62-9 pubmed publisher
    ..Pre-polycythemic stages of polycythemia vera with a low hemoglobin level at onset are diagnosed by positive JAK2V617F mutation status, a low ..
  38. Tefferi A. Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management. Am J Hematol. 2011;86:292-301 pubmed publisher
    b>Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively...
  39. Crisà E, Venturino E, Passera R, Prina M, Schinco P, Borchiellini A, et al. A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs. Ann Hematol. 2010;89:691-9 pubmed publisher
    The clinical impact of polycythemia vera (PV) diagnostic and therapeutic guidelines is still undetermined. In particular, the recommended target of hematocrit (Hct) <0...
  40. Guglielmelli P, Biamonte F, Score J, Hidalgo Curtis C, Cervantes F, Maffioli M, et al. EZH2 mutational status predicts poor survival in myelofibrosis. Blood. 2011;118:5227-34 pubmed publisher
    ..We conclude that EZH2 mutations are independently associated with shorter survival in patients with PMF...
  41. Tam C, Kantarjian H, Cortes J, Lynn A, Pierce S, Zhou L, et al. Dynamic model for predicting death within 12 months in patients with primary or post-polycythemia vera/essential thrombocythemia myelofibrosis. J Clin Oncol. 2009;27:5587-93 pubmed publisher
    ..We aimed to define an accelerated phase (AP) in MF by characterizing disease features that can identify patients with median overall survival of <or= 12 months at any time in the disease course...
  42. Guglielmelli P, Tozzi L, Bogani C, Iacobucci I, Ponziani V, Martinelli G, et al. Overexpression of microRNA-16-2 contributes to the abnormal erythropoiesis in polycythemia vera. Blood. 2011;117:6923-7 pubmed publisher
    ..Here, we show that mature miR-16 levels are abnormally increased in CD34(+) cells of patients with polycythemia vera as a consequence of preferential expression of miR-16-2 on chromosome 3 rather than of miR-16-1 on ..
  43. Randi M, Ruzzon E, Tezza F, Scapin M, Duner E, Scandellari R, et al. JAK2V617F mutation is common in old patients with polycythemia vera and essential thrombocythemia. Aging Clin Exp Res. 2011;23:17-21 pubmed
    JAK2V617F mutation occurs in 90% of polycythemia vera (PV) and in 50% of essential thrombocythemia (ET) patients.
  44. Bonicelli G, Abdulkarim K, Mounier M, Johansson P, Rossi C, Jooste V, et al. Leucocytosis and thrombosis at diagnosis are associated with poor survival in polycythaemia vera: a population-based study of 327 patients. Br J Haematol. 2013;160:251-4 pubmed publisher
    ..Age and leucocyte count are the main predicting factors for survival in polycythaemia vera...
  45. Skov V, Larsen T, Thomassen M, Riley C, Jensen M, Bjerrum O, et al. Molecular profiling of peripheral blood cells from patients with polycythemia vera and related neoplasms: identification of deregulated genes of significance for inflammation and immune surveillance. Leuk Res. 2012;36:1387-92 pubmed publisher
    Essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) are hematopoietic stem cell neoplasms that may be associated with autoimmune or chronic inflammatory disorders...
  46. Jones A, Chase A, Silver R, Oscier D, Zoi K, Wang Y, et al. JAK2 haplotype is a major risk factor for the development of myeloproliferative neoplasms. Nat Genet. 2009;41:446-9 pubmed publisher
    ..More than 95% of cases of polycythemia vera, and roughly half of essential thrombocythemia and primary myelofibrosis acquire a unique somatic 1849G>T ..
  47. Ma A, Fan A, Ward A, Liongue C, Lewis R, Cheng S, et al. A novel zebrafish jak2a(V581F) model shared features of human JAK2(V617F) polycythemia vera. Exp Hematol. 2009;37:1379-1386.e4 pubmed publisher
    ..A gain-of-function JAK2 (JAK2(V617F)) mutation in human is pathogenetically linked to polycythemia vera (PV)...
  48. Mullally A, Bruedigam C, Poveromo L, Heidel F, Purdon A, Vu T, et al. Depletion of Jak2V617F myeloproliferative neoplasm-propagating stem cells by interferon-? in a murine model of polycythemia vera. Blood. 2013;121:3692-702 pubmed publisher
  49. Silver R, Kiladjian J, Hasselbalch H. Interferon and the treatment of polycythemia vera, essential thrombocythemia and myelofibrosis. Expert Rev Hematol. 2013;6:49-58 pubmed publisher
    ..Now, low-dose rIFN-? is stressed, starting relatively early in the course of the MPNs. In polycythemia vera, this has resulted in a significant clinical, hematologic, morphologic and molecular response manifested by ..
  50. Passamonti F, Rumi E, Pietra D, Elena C, Boveri E, Arcaini L, et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia. 2010;24:1574-9 pubmed publisher
    ..JAK2 (V617F) mutant allele burden and clinical phenotype, disease progression and survival in patients with polycythemia vera (PV)...
  51. Yuan S, Shinfeld A, Raanani E. Massive intraventricular thrombus in polycythemia vera. J Card Surg. 2009;24:110-2 pubmed publisher
    b>Polycythemia vera is a chronic myeloproliferative disorder which is prone to thrombotic complications. However, thrombosis of the cardiovascular system is an extremely rare complication of polycythemia vera...
  52. Mesa R, Tefferi A. Emerging drugs for the therapy of primary and post essential thrombocythemia, post polycythemia vera myelofibrosis. Expert Opin Emerg Drugs. 2009;14:471-9 pubmed publisher
    ..patients with myelofibrosis (MF) (both those with primary myelofibrosis or having evolved from an antecedent polycythemia vera or essential thrombocythemia) present many challenges to the hematologist...
  53. Marubayashi S, Koppikar P, Taldone T, Abdel Wahab O, West N, Bhagwat N, et al. HSP90 is a therapeutic target in JAK2-dependent myeloproliferative neoplasms in mice and humans. J Clin Invest. 2010;120:3578-93 pubmed publisher
    ..report here that an HSP90 inhibitor, PU-H71, demonstrated efficacy in cell line and mouse models of the MPN polycythemia vera (PV) and essential thrombocytosis (ET) by disrupting JAK2 protein stability...
  54. Barbui T, Carobbio A, Finazzi G, Vannucchi A, Barosi G, Antonioli E, et al. Inflammation and thrombosis in essential thrombocythemia and polycythemia vera: different role of C-reactive protein and pentraxin 3. Haematologica. 2011;96:315-8 pubmed publisher
    ..3 might be correlated with cardiovascular complications in patients with essential thrombocythemia and polycythemia vera. High sensitivity C-reactive protein and pentraxin 3 were measured in 244 consecutive essential ..
  55. Abe M, Funakoshi Tago M, Tago K, Kamishimoto J, Aizu Yokota E, Sonoda Y, et al. The polycythemia vera-associated Jak2 V617F mutant induces tumorigenesis in nude mice. Int Immunopharmacol. 2009;9:870-7 pubmed publisher
    The somatic Jak2 mutation (V617F) was identified in most patients with polycythemia vera (PV). Here, we show that the activating Jak2 V617F mutant completely protected Ba/F3 cells from cytokine withdrawal-induced apoptotic cell death...
  56. Mathur A, Mo J, Kraus M, O Hare E, Sinclair P, Young J, et al. An inhibitor of Janus kinase 2 prevents polycythemia in mice. Biochem Pharmacol. 2009;78:382-9 pubmed publisher
    b>Polycythemia vera (PV) is a myeloproliferative disorder characterized by increased red cell mass and splenomegaly in the absence of secondary causes [Tefferi A., Spivak J.L., Polycythemia vera: scientific advances and current practice...
  57. Zhan H, Spivak J. The diagnosis and management of polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the JAK2 V617F era. Clin Adv Hematol Oncol. 2009;7:334-42 pubmed
    The chronic myeloproliferative disorders, polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are clonal stem cell disorders that occur at a low frequency and mimic not only each other clinically, but also many ..
  58. Kraus M, Wang Y, Aleksandrowicz D, Bachman E, Szewczak A, Walker D, et al. Efficacious intermittent dosing of a novel JAK2 inhibitor in mouse models of polycythemia vera. PLoS ONE. 2012;7:e37207 pubmed publisher
    A high percentage of patients with the myeloproliferative disorder polycythemia vera (PV) harbor a Val617?Phe activating mutation in the Janus kinase 2 (JAK2) gene, and both cell culture and mouse models have established a functional ..
  59. Zhan H, Cardozo C, Yu W, Wang A, Moliterno A, Dang C, et al. MicroRNA deregulation in polycythemia vera and essential thrombocythemia patients. Blood Cells Mol Dis. 2013;50:190-5 pubmed publisher
    b>Polycythemia vera (PV) and essential thrombocythemia (ET) are the two most common myeloproliferative neoplasms...
  60. Akada H, Akada S, Gajra A, Bair A, Graziano S, Hutchison R, et al. Efficacy of vorinostat in a murine model of polycythemia vera. Blood. 2012;119:3779-89 pubmed publisher
    ..inhibitor of histone deacetylase, against cells expressing JAK2V617F and in an animal model of polycythemia vera (PV)...
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    ..reported herein a case of successful urokinase intra-arterial thrombolytic treatment for HAT in an essential polycythemia vera patient following pancreato-biliary surgery...
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    ..The current study clarifies the contribution of specific disease and host characteristics to the risk of arterial versus venous thrombosis in essential thrombocythemia...
  63. Quintas Cardama A, Abdel Wahab O, Manshouri T, Kilpivaara O, Cortes J, Roupie A, et al. Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon ?-2a. Blood. 2013;122:893-901 pubmed publisher
    ..2a (PEG-IFN-?-2a) has previously been shown to induce hematologic and molecular responses in patients with polycythemia vera (PV) or essential thrombocythemia (ET)...
  64. Barbui T, Carobbio A, Rambaldi A, Finazzi G. Perspectives on thrombosis in essential thrombocythemia and polycythemia vera: is leukocytosis a causative factor?. Blood. 2009;114:759-63 pubmed publisher
    ..identified as an independent predictor of major thrombosis in both essential thrombocythemia (ET) and polycythemia vera (PV)...
  65. Kiladjian J, Chevret S, Dosquet C, Chomienne C, Rain J. Treatment of polycythemia vera with hydroxyurea and pipobroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011;29:3907-13 pubmed publisher
    The overall impact of hydroxyurea (HU) or pipobroman treatments on the long-term outcome of patients with polycythemia vera (PV) has not been assessed in randomized studies...