Skip to main content

Advertisement

SpringerLink
Log in

Analysis of thrombosis and bleeding complications in patients with polycythemia vera: a Turkish retrospective study

  • Original Article
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

The aims of this study are to determine the incidence and risk factors of thrombosis and bleeding in polycythemia vera (PV) patients and to research the effects of these risk factors on survival. The medical records of 155 PV patients were analyzed retrospectively. Patients were divided into groups according to whether or not thrombosis had developed in follow-up, and according to whether or not bleeding had occurred during follow-up. The mean age at diagnosis was 53 years, and the mean follow-up period was 66 months. The percentage of cases in which thrombosis events had occurred before diagnosis and during follow-up were 26 and 28 %, respectively. Comparisons of disease duration and average thrombosis risk score between groups with or without thrombosis drew statistically significant results. A patient’s history of thrombosis and thrombocytosis at first visit was found to have a significant effect on thrombosis recurrence. The major bleeding rate was 8 %. Post-PV myelofibrosis was an independent risk factor for bleeding. The major cause of death among the patients in this study was primary thrombosis. The most important causes of mortality among PV patients are thrombosis, and the most prominent risk factors for thrombosis development are disease duration and high thrombosis risk scores. Thrombocytosis in patients with a history of thrombosis may cause thrombosis recurrence during the follow-up period.

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

  1. Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol. 2005;128(3):275–90.

    Article  CAS  PubMed  Google Scholar 

  2. Polycythemia vera: the natural history of 1213 patients followed for 20 years. Gruppo Italiano Studio Policitemia. Ann Intern Med. 1995;123(9):656–64.

    Article  Google Scholar 

  3. Finazzi G, Rambaldi A, Guerini V, Carobbo A, Barbui T. Risk of thrombosis in patients with essential thrombocythemia and polycythemia vera according to JAK2V617F mutation status. Haematologica. 2007;92(1):135–6.

    Article  PubMed  Google Scholar 

  4. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. Am J Hematol. 2015;90(2):162–73 (Erratum in: Am J Hematol. 2015 Sep;90(9):849).

    Article  CAS  PubMed  Google Scholar 

  5. Lim Y, Lee JO, Kim SH, Kim JW, Kim YJ, Lee KW, et al. Prediction of thrombotic and hemorrhagic events during polycythemia vera or essential thrombocythemia based on leukocyte burden. Thromb Res. 2015;135(5):846–51.

    Article  CAS  PubMed  Google Scholar 

  6. Marchioli R, Finazzi G, Specchia G, Cacciola R, Cavazzina R, Cilloni D, et al. CYTO-PV Collaborative Group. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl JMed. 2013;368(1):22–33.

    Article  CAS  Google Scholar 

  7. Landolfi R, DiGennaro L. Prevention of thrombosis in polycythemia vera and essential thrombocythemia. Haematologica. 2008;93(3):331–5.

    Article  CAS  PubMed  Google Scholar 

  8. Kander EM, Raza S, Zhou Z, Gao J, Zakarija A, McMahon BJ, et al. Bleeding complications in BCR-ABL negative myeloproliferative neoplasms: prevalence, type, and risk factors in a single-center cohort. Int J Hematol. 2015;102(5):587–93.

    Article  CAS  PubMed  Google Scholar 

  9. Budde U, vanGenderen PJ. Acquired vonWillebrand disease in patients with high platelet counts. Semin Thromb Hemost. 1997;23(5):425–31.

    Article  CAS  PubMed  Google Scholar 

  10. Pearson TC, Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1996;22(11):87–93.

    PubMed  Google Scholar 

  11. Thiele J, Kvasnicka HM. [Chronic myeloproliferative disorders. The new WHO classification]. Pathologe. 2001;22(6):429–43.

    Article  CAS  PubMed  Google Scholar 

  12. Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22(1):14–22.

    Article  CAS  PubMed  Google Scholar 

  13. Skoda R. The genetic basis of myeloproliferative disorders. Hematology Am Soc Hematol Educ Program. 2007;1–10.

  14. Tefferi A. Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia. 2010;24(6):1128–38.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F, et al. In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology. Blood. 2014;124(19):3021–3.

    Article  CAS  PubMed  Google Scholar 

  16. Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23(10):2224–32.

    Article  PubMed  Google Scholar 

  17. 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(7):691–9.

    Article  PubMed  Google Scholar 

  18. Denninger MH, Chaït Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, Erlinger S, Briere J, Valla D. Cause of portal orhepaticvenousthrombosis inadults: the role of multipleconcurrentfactors. Hepatology. 2000;31(3):587–91.

    Article  CAS  PubMed  Google Scholar 

  19. Cardin F, Graffeo M, McCormick PA, McIntyre N, Burroughs A. Adult, “idiopathic” extrahepatic venous thrombosis. Importance of putative “latent” myeloproliferative disorders and comparison with cases with known etiology. Dig Dis Sci. 1992;37(3):335–9.

    Article  CAS  PubMed  Google Scholar 

  20. Bazzan M, Tamponi G, Schinco P, Vaccarino A, Foli C, Gallone G, et al. Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia. Ann Hematol. 1999;78(12):539–43.

    Article  CAS  PubMed  Google Scholar 

  21. Varma S, Sharma A, Malhotra P, Kumari S, Jain S, Varma N. Thrombotic complications of polycythemia vera. Hematology. 2008;13(6):319–23.

    Article  CAS  PubMed  Google Scholar 

  22. De Stefano V, Za T, Rossi E, Vannucchi AM, Ruggeri M, Elli E, et al. GIMEMA CMD-Working Party. Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments. Haematologica. 2008;93(3):372–80.

    Article  PubMed  Google Scholar 

  23. Landolfi R, Di Gennaro L, Barbui T, De Stefano V, Finazzi G, Marfisi R, et al. European Collaboration on Low-Dose Aspirin in Polycythemia Vera (ECLAP). Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood. 2007;109(6):2446–52.

    Article  CAS  PubMed  Google Scholar 

  24. Pearson TC. The risk of thrombosis in essential thrombocythemia and polycythemia vera. Semin Oncol. 2002;29(3):16–21.

    Article  PubMed  Google Scholar 

  25. Di Nisio M, Barbui T, Di Gennaro L, Borrelli G, Finazzi G, Landolfi R, et al. European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Investigators. The haematocrit and platelet target in polycythemia vera. Br J Haematol. 2007;136(2):249–59.

    Article  PubMed  Google Scholar 

  26. Passamonti F, Rumi E. Clinical relevance of JAK2 (V617F) mutant allele burden. Haematologica. 2009;94(1):7–10.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Deininger M, Radich J, Burn TC, Huber R, Paranagama D, Verstovsek S. The effect of long-term ruxolitinib treatment on JAK2p.V617F allele burden in patients with myelofibrosis. Blood. 2015;126(13):1551–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Vannucchi AM. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015;372(17):1670–1.

    Article  PubMed  Google Scholar 

  29. Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, et al. European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004;350(2):114–24.

    Article  CAS  PubMed  Google Scholar 

  30. Finazzi G, Barbui T. How I treat patients with polycythemia vera. Blood. 2007;109(12):5104–11.

    Article  CAS  PubMed  Google Scholar 

  31. Tefferi A, Barbui T. New and treatment-relevant risk stratification for thrombosis in essential thrombocythemia and polycythemia vera. Am J Hematol. 2015;90(8):683–5.

    Article  PubMed  Google Scholar 

  32. Chıevıtz E, Thıede T. Complications and causes of death in polycythaemia vera. Acta Med Scand. 1962;172:513–23.

    PubMed  Google Scholar 

  33. Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. N Engl J Med. 1981;304(8):441–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Okmeydanı Training and Research Hospital, Istanbul University, Istanbul, Turkey

    A. M. Yesilova & I. D. Toprak

  2. Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Cerrahpasa, 34303, Istanbul, Turkey

    S. Yavuzer, H. Yavuzer & M. Cengiz

  3. Student of Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey

    E. Hanedar

  4. Department of Hematology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey

    M. C. Ar & Z. Baslar

Authors
  1. A. M. Yesilova
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. S. Yavuzer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Yavuzer
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. Cengiz
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. I. D. Toprak
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. E. Hanedar
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. M. C. Ar
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Z. Baslar
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to S. Yavuzer.

Ethics declarations

Conflict of interest

All authors declare that they have no conflict of interest.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yesilova, A.M., Yavuzer, S., Yavuzer, H. et al. Analysis of thrombosis and bleeding complications in patients with polycythemia vera: a Turkish retrospective study. Int J Hematol 105, 70–78 (2017). https://doi.org/10.1007/s12185-016-2105-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-016-2105-0

Keywords

Search

Navigation