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Austrian recommendations for the management of polycythemia vera

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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.

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Acknowledgements

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

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Authors and Affiliations

  1. Department of Internal Medicine IV, Klinikum Wels-Grieskirchen, Wels, Austria

    Sonja Burgstaller

  2. Department of Internal Medicine I, Ordensklinikum Linz Elisabethinen, Linz, Austria

    Veronika Buxhofer-Ausch

  3. 3rd Medical Department, Hanusch Hospital, Vienna, Austria

    Thamer Sliwa

  4. Institute of Pathology, Medical University Graz, Graz, Austria

    Christine Beham-Schmid

  5. Division of Hematology and Oncology, Innsbruck Medical University, Innsbruck, Austria

    Günther Gastl

  6. 5th Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Vienna, Austria

    Klaus Geissler

  7. 3rd Medical Department with Hematology and Medical Oncology, Hemostaseology, Rheumatology and Infectiology, Laboratory for Immunological and Molecular Cancer Research, Paracelsus Medical University Hospital Salzburg, Salzburg, Austria

    Thomas Melchardt

  8. Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria

    Maria Krauth

  9. Department of Internal Medicine with Hematology and Oncology, Steiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-Fürstenfeld, Fürstenfeld, Austria

    Peter Krippl

  10. Department of Internal Medicine I, Ordensklinikum Barmherzige Schwestern, Linz, Austria

    Andreas Petzer

  11. Department of Internal Medicine II, Landeskrankenhaus Feldkirch, Feldkirch, Austria

    Holger Rumpold

  12. Division of Hematology, Medical University of Graz, Graz, Austria

    Albert Wölfler

  13. Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria

    Heinz Gisslinger

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  1. Sonja Burgstaller
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  10. Andreas Petzer
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  11. Holger Rumpold
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  13. Heinz Gisslinger
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Corresponding author

Correspondence to Sonja Burgstaller.

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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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Burgstaller, S., Buxhofer-Ausch, V., Sliwa, T. et al. Austrian recommendations for the management of polycythemia vera. Wien Klin Wochenschr 130, 535–542 (2018). https://doi.org/10.1007/s00508-018-1359-3

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