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Wiener klinische Wochenschrift

, Volume 130, Issue 17–18, pp 535–542 | Cite as

Austrian recommendations for the management of polycythemia vera

  • Sonja BurgstallerEmail author
  • Veronika Buxhofer-Ausch
  • Thamer Sliwa
  • Christine Beham-Schmid
  • Günther Gastl
  • Klaus Geissler
  • Thomas Melchardt
  • Maria Krauth
  • Peter Krippl
  • Andreas Petzer
  • Holger Rumpold
  • Albert Wölfler
  • Heinz Gisslinger
original article
  • 214 Downloads

Summary

Polycythemia vera (PV) is a clonal disease arising from hematopoietic stem cells. Erythrocytosis is the hallmark of the disease but leukocytosis, thrombocytosis and splenomegaly may also be present. Thromboembolic complications occur in about 20% of patients. Circulatory disturbances as well as pruritus represent frequent symptoms of the disease. Mutations in the JAK2 gene are present in 95% of patients in exon 14 (V617F) and in 3% in exon 12. The main goal of the treatment for patients with PV is the prevention of thromboembolic events, transformation to myelofibrosis and acute myeloid leukemia. Interferon alpha and hydroxyurea are used as first-line treatment for high risk patients. For patients unresponsive to first-line therapy ruxolitinib is available.

Keywords

Polycythemia vera PV Management recommendations Risk stratification Treatment 

Notes

Acknowledgements

A single meeting for discussion of the final version of these recommendations was sponsored by Novartis Pharma GmbH and AOP Orphan Pharmaceuticals AG.

Conflict of interest

The following authors declare that they have served as consultants or received speakers fees from industrial companies: S. Burgstaller Novartis, AOP Orphan, K. Geissler Novartis, AOP Orphan, H. Gisslinger Novartis, AOP Orphan, Baxalta, Celgene, M. Krauth Novartis, AOP Orphan, A. Wölfler Novartis, AOP. V. Buxhofer-Ausch, T. Sliwa, C. Beham-Schmid, G. Gastl, T. Melchardt, P. Krippl, A. Petzer and H. Rumpold declare that they have no competing interests.

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Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2018

Authors and Affiliations

  • Sonja Burgstaller
    • 1
    Email author
  • Veronika Buxhofer-Ausch
    • 2
  • Thamer Sliwa
    • 3
  • Christine Beham-Schmid
    • 4
  • Günther Gastl
    • 5
  • Klaus Geissler
    • 6
  • Thomas Melchardt
    • 7
  • Maria Krauth
    • 8
  • Peter Krippl
    • 9
  • Andreas Petzer
    • 10
  • Holger Rumpold
    • 11
  • Albert Wölfler
    • 12
  • Heinz Gisslinger
    • 13
  1. 1.Department of Internal Medicine IVKlinikum Wels-GrieskirchenWelsAustria
  2. 2.Department of Internal Medicine IOrdensklinikum Linz ElisabethinenLinzAustria
  3. 3.3rd Medical DepartmentHanusch HospitalViennaAustria
  4. 4.Institute of PathologyMedical University GrazGrazAustria
  5. 5.Division of Hematology and OncologyInnsbruck Medical UniversityInnsbruckAustria
  6. 6.5th Medical Department with Hematology, Oncology and Palliative MedicineHospital HietzingViennaAustria
  7. 7.3rd Medical Department with Hematology and Medical Oncology, Hemostaseology, Rheumatology and Infectiology, Laboratory for Immunological and Molecular Cancer ResearchParacelsus Medical University Hospital SalzburgSalzburgAustria
  8. 8.Department of Internal Medicine I, Division of Hematology and Blood CoagulationMedical University of ViennaViennaAustria
  9. 9.Department of Internal Medicine with Hematology and OncologySteiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-FürstenfeldFürstenfeldAustria
  10. 10.Department of Internal Medicine IOrdensklinikum Barmherzige SchwesternLinzAustria
  11. 11.Department of Internal Medicine IILandeskrankenhaus FeldkirchFeldkirchAustria
  12. 12.Division of HematologyMedical University of GrazGrazAustria
  13. 13.Department of Internal Medicine I, Division of Hematology and Blood CoagulationMedical University of ViennaViennaAustria

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