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Austrian recommendations for the management of primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis: an expert statement

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Summary

The entity “myelofibrosis” represents a subgroup of the Philadelphia chromosome-negative myeloproliferative neoplasms. It comprises primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis. This heterogeneous disease is characterized by clonal myeloproliferation, dysregulated kinase signalling and the abnormal expression of several proinflammatory cytokines. Clinically, patients present with symptoms related to thrombocytosis/leukocytosis, anemia and/or progressive splenomegaly. Mutations in Janus kinase 2, an enzyme that is essential for the normal development of erythrocytes, granulocytes, and platelets, notably the V617F mutation, have been identified in approximately 60% of patients with primary myelofibrosis. Recent molecular advances have not only elucidated critical pathways in the pathogenesis of the disease, but also contributed to a more precise assessment of a patient’s individual risk. While allogeneic stem cell transplantation remains the only curative treatment, the natural course of the disease and the patient’s survival and quality of life may be improved by new treatments, notably ruxolitinib, the first Janus kinase 1/2 inhibitor approved for the management of myelofibrosis. Additional treatment options are being explored.

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Acknowledgements

The development of these recommendations was supported by Novartis Pharma GmbH, Austria including funding of a medical writer. The authors did not receive financial compensation from Novartis Pharma GmbH for authoring the manuscript.

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Authors and Affiliations

  1. 3rd Medical Department, Hanusch Hospital, Vienna, Austria

    Thamer Sliwa

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

    Christine Beham-Schmid

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

    Sonja Burgstaller

  4. Department of Internal Medicine I, Elisabethinen Hospital Linz, Linz, Austria

    Veronika Buxhofer-Ausch

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

    Maria Krauth & Heinz Gisslinger

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

    Peter Krippl

  9. Department of Internal Medicine, LKH Feldkirch, Feldkirch, Austria

    Alois Lang

  10. Department of Medical Oncology, Hematology and Gastroenterology, Barmherzige Schwestern Hospital Linz, Linz, Austria

    Andreas Petzer

  11. Department of Oncology, Landesklinikum Wiener Neustadt, Wiener Neustadt, Austria

    Stefan Wöhrer

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

    Albert Wölfler

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  1. Thamer Sliwa
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Correspondence to Thamer Sliwa.

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Conflict of interest

The following authors declare that they have served as consultants or received financial support for performing a research project of industrial companies: S. Burgstaller: Novartis, AOP Orphan, Celgene; K. Geissler: Novartis; A. Lang: Novartis; A. Petzer: Novartis; A. Wölfler: Novartis; H. Gisslinger: Novartis, AOP Orphan, Baxalta, Celgene. T. Sliwa, C. Beham-Schmid, V. Buxhofer-Ausch, G. Gastl, M. Krauth, P. Krippl and S. Wöhrer declare that they have no competing interests.

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Sliwa, T., Beham-Schmid, C., Burgstaller, S. et al. Austrian recommendations for the management of primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis: an expert statement. Wien Klin Wochenschr 129, 293–302 (2017). https://doi.org/10.1007/s00508-016-1120-8

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