Skip to main content

Advertisement

SpringerLink
Log in

New and old prognostic factors in polycythemia vera

  • Published:
Current Hematologic Malignancy Reports Aims and scope Submit manuscript

Abstract

Polycythemia vera is a chronic myeloproliferative disorder affecting people in the sixth decade of life. The disease is considered long-lasting; median survival exceeds 18 years. As thrombosis is the main complication during follow-up, scoring systems have focused on the risk of thrombosis. Age over 60 years and prior thrombosis are considered the standard risk factors for thrombosis and represent the state of the art for risk stratification in decision making regarding treatment. However, new disease-based risk factors—leukocytosis and the JAK2V617F mutation burden—seem to be emerging as indicators of prognosis in polycythemia vera. Leukocytosis, as a marker of disease proliferation, seems to have an impact on thrombosis, post-polycythemia vera myelofibrosis, leukemia, and survival. The role of the JAK2V617F mutation as a prognostic factor requires further validation, but it may have a dominant role in disease progression and in the activation of platelets and leukocytes.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Passamonti F, Rumi E, Pungolino E, et al.: Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med 2004, 117:755–761.

    Article  PubMed  Google Scholar 

  2. Passamonti F, Malabarba L, Orlandi E, et al.: Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia. Haematologica 2003, 88:13–18.

    PubMed  Google Scholar 

  3. Polycythemia vera: the natural history of 1213 patients followed for 20 years. Gruppo Italiano Studio Policitemia. Ann Intern Med 1995, 123:656–664.

  4. Kralovics R, Passamonti F, Buser AS, et al.: A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 2005, 352:1779–1790.

    Article  PubMed  CAS  Google Scholar 

  5. Pietra D, Li S, Brisci A, et al.: Somatic mutations of JAK2 exon 12 in patients with JAK2 (V617F)-negative myeloproliferative disorders. Blood 2008, 111:1686–1689.

    Article  PubMed  CAS  Google Scholar 

  6. Scott LM, Tong W, Levine RL, et al.: JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med 2007, 356:459–468.

    Article  PubMed  CAS  Google Scholar 

  7. Landolfi R, Di Gennaro L, Barbui T, et al.: Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 2007, 109:2446–2452.

    Article  PubMed  CAS  Google Scholar 

  8. Passamonti F, Rumi E, Caramella M, et al.: A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis. Blood 2008, 111:3383–3387.

    Article  PubMed  CAS  Google Scholar 

  9. Gangat N, Strand J, Li CY, et al.: Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation. Br J Haematol 2007, 138:354–358.

    Article  PubMed  Google Scholar 

  10. Rozman C, Giralt M, Feliu E, et al.: Life expectancy of patients with chronic nonleukemic myeloproliferative disorders. Cancer 1991, 67:2658–2663.

    Article  PubMed  CAS  Google Scholar 

  11. Kiladjian JJ, Gardin C, Renoux M, et al.: Long-term outcomes of polycythemia vera patients treated with pipobroman as initial therapy. Hematol J 2003, 4:198–207.

    Article  PubMed  CAS  Google Scholar 

  12. Ania BJ, Suman VJ, Sobell JL, et al.: Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935–1989. Am J Hematol 1994, 47:89–93.

    Article  PubMed  CAS  Google Scholar 

  13. Najean Y, Mugnier P, Dresch C, Rain JD: Polycythaemia vera in young people: an analysis of 58 cases diagnosed before 40 years. Br J Haematol 1987, 67:285–291.

    Article  PubMed  CAS  Google Scholar 

  14. Perea G, Remacha A, Besses C, et al.: Is polycythemia vera a serious disease in young adults? Haematologica 2001, 86:543–544.

    PubMed  CAS  Google Scholar 

  15. Marchioli R, Finazzi G, Landolfi R, et al.: Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 2005, 23:2224–2232.

    Article  PubMed  Google Scholar 

  16. Mesa RA, Verstovsek S, Cervantes F, et al.: Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the International Working Group for Myelofibrosis Research And Treatment (IWG-MRT). Leuk Res 2007, 31:737–740.

    Article  PubMed  Google Scholar 

  17. Barosi G, Mesa RA, Thiele J, et al.: Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment. Leukemia 2008, 22:437–438.

    Article  PubMed  CAS  Google Scholar 

  18. Passamonti F, Rumi E, Pietra D, et al.: Relation between JAK2 (V617F) mutation status, granulocyte activation, and constitutive mobilization of CD34+ cells into peripheral blood in myeloproliferative disorders. Blood 2006, 107:3676–3682.

    Article  PubMed  CAS  Google Scholar 

  19. Passamonti F, Vanelli L, Malabarba L, et al.: Clinical utility of the absolute number of circulating CD34-positive cells in patients with chronic myeloproliferative disorders Haematologica 2003, 88:1123–1129.

    PubMed  Google Scholar 

  20. Deeg HJ, Gooley TA, Flowers ME, et al.: Allogeneic hematopoietic stem cell transplantation for myelofibrosis. Blood 2003, 102:3912–3918.

    Article  PubMed  CAS  Google Scholar 

  21. Rondelli D, Barosi G, Bacigalupo A, et al.: Allogeneic hematopoietic stem-cell transplantation with reducedintensity conditioning in intermediate- or high-risk patients with myelofibrosis with myeloid metaplasia. Blood 2005, 105:4115–4119.

    Article  PubMed  CAS  Google Scholar 

  22. Pardanani A: JAK2 inhibitor therapy in myeloproliferative disorders: rationale, preclinical studies and ongoing clinical trials. Leukemia 2008, 22:23–30.

    Article  PubMed  CAS  Google Scholar 

  23. Passamonti F, Rumi E, Arcaini L, et al.: Leukemic transformation of polycythemia vera: a single center study of 23 patients. Cancer 2005, 104:1032–1036.

    Article  PubMed  Google Scholar 

  24. Vardiman JW, Harris NL, Brunning RD: The World Health Organization (WHO) classification of the myeloid neoplasms Blood 2002, 100:2292–2302.

    Article  PubMed  CAS  Google Scholar 

  25. Tam CS, Nussenzveig RM, Popat U, et al.: The natural history and treatment outcome of blast phase BCRABL [negative] myeloproliferative neoplasms. Blood 2008, 112:1628–1637.

    Article  PubMed  CAS  Google Scholar 

  26. Elliott MA, Tefferi A: Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol 2005, 128:275–290.

    Article  PubMed  CAS  Google Scholar 

  27. Passamonti F, Brusamolino E, Lazzarino M, et al.: Efficacy of pipobroman in the treatment of polycythemia vera: long-term results in 163 patients. Haematologica 2000, 85:1011–1018.

    PubMed  CAS  Google Scholar 

  28. Berk PD, Goldberg JD, Donovan PB, et al.: Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol 1986, 23:132–143.

    PubMed  CAS  Google Scholar 

  29. Landolfi R, Marchioli R, Kutti J, et al.: Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med 2004, 350:114–124.

    Article  PubMed  CAS  Google Scholar 

  30. Di Nisio M, Barbui T, Di Gennaro L, et al.: The haematocrit and platelet target in polycythemia vera. Br J Haematol 2007, 136:249–259.

    Article  PubMed  Google Scholar 

  31. Falanga A, Marchetti M, Evangelista V, et al.: Polymorphonuclear leukocyte activation and hemostasis in patients with essential thrombocythemia and polycythemia vera. Blood 2000, 96:4261–4266.

    PubMed  CAS  Google Scholar 

  32. Falanga A, Marchetti M, Vignoli A, et al.: V617F JAK-2 mutation in patients with essential thrombocythemia: relation to platelet, granulocyte, and plasma hemostatic and inflammatory molecules. Exp Hematol 2007, 35:702–711.

    Article  PubMed  CAS  Google Scholar 

  33. Arellano-Rodrigo E, Alvarez-Larran A, Reverter JC, et al.: Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status. Haematologica 2006, 91:169–175.

    PubMed  CAS  Google Scholar 

  34. Vannucchi AM, Antonioli E, Guglielmelli P, et al.: Clinical profile of homozygous JAK2 617V>F mutation in patients with polycythemia vera or essential thrombocythemia. Blood 2007, 110:840–846.

    Article  PubMed  CAS  Google Scholar 

  35. Tefferi A, Lasho TL, Schwager SM, et al.: The clinical phenotype of wild-type, heterozygous, and homozygous JAK2V617F in polycythemia vera. Cancer 2006, 106:631–635.

    Article  PubMed  CAS  Google Scholar 

  36. Vannucchi AM, Antonioli E, Guglielmelli P, et al.: Prospective identification of high-risk polycythemia vera patients based on JAK2(V617F) allele burden. Leukemia 2007, 21:1952–1959.

    Article  PubMed  CAS  Google Scholar 

  37. Tefferi A, Strand JJ, Lasho TL, et al.: Bone marrow JAK2V617F allele burden and clinical correlates in polycythemia vera. Leukemia 2007, 21:2074–2075.

    Article  PubMed  CAS  Google Scholar 

  38. Lacout C, Pisani DF, Tulliez M, et al.: JAK2V617F expression in murine hematopoietic cells leads to MPD mimicking human PV with secondary myelofibrosis. Blood 2006, 108:1652–1660.

    Article  PubMed  CAS  Google Scholar 

  39. Passamonti F, Rumi E, Pietra D, et al.: JAK2 (V617F) mutation burden, phenotype, and clinical course of chronic myeloproliferative disorders [abstract]. Haematologica 2007, 92(s1):243.

    Google Scholar 

  40. Finazzi G, Caruso V, Marchioli R, et al.: Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study. Blood 2005, 105:2664–2670.

    Article  PubMed  CAS  Google Scholar 

  41. Rumi E, Passamonti F, Della Porta MG, et al.: Familial chronic myeloproliferative disorders: clinical phenotype and evidence of disease anticipation. J Clin Oncol 2007, 25:5630–5635.

    Article  PubMed  Google Scholar 

  42. Theocharides A, Boissinot M, Girodon F, et al.: Leukemic blasts in transformed JAK2-V617F-positive myeloproliferative disorders are frequently negative for the JAK2-V617F mutation. Blood 2007, 110:375–379.

    Article  PubMed  CAS  Google Scholar 

  43. Campbell PJ, Baxter EJ, Beer PA, et al.: Mutation of JAK2 in the myeloproliferative disorders: timing, clonality studies, cytogenetic associations, and role in leukemic transformation. Blood 2006, 108:3548–3555.

    Article  PubMed  CAS  Google Scholar 

  44. Gangat N, Strand J, Lasho TL, et al.: Cytogenetic studies at diagnosis in polycythemia vera: clinical and JAK2V617F allele burden correlates. Eur J Haematol 2008, 80:197–200.

    Article  PubMed  Google Scholar 

  45. Spivak JL: Polycythemia vera: myths, mechanisms, and management. Blood 2002, 100:4272–4290.

    Article  PubMed  CAS  Google Scholar 

  46. Dingli D, Schwager SM, Mesa RA, et al.: Presence of unfavorable cytogenetic abnormalities is the strongest predictor of poor survival in secondary myelofibrosis. Cancer 2006, 106:1985–1989.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematology, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Viale Golgi 19, 27100, Pavia, Italy

    Francesco Passamonti

Authors
  1. Francesco Passamonti
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Francesco Passamonti.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Passamonti, F. New and old prognostic factors in polycythemia vera. Curr Hematol Malig Rep 4, 19–24 (2009). https://doi.org/10.1007/s11899-009-0003-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11899-009-0003-8

Keywords

Search

Navigation