Wiener klinische Wochenschrift

, Volume 129, Issue 9–10, pp 293–302 | Cite as

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

  • Thamer SliwaEmail author
  • Christine Beham-Schmid
  • Sonja Burgstaller
  • Veronika Buxhofer-Ausch
  • Günther Gastl
  • Klaus Geissler
  • Maria Krauth
  • Peter Krippl
  • Alois Lang
  • Andreas Petzer
  • Stefan Wöhrer
  • Albert Wölfler
  • Heinz Gisslinger
consensus report


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.


Primary myelofibrosis Post-polycythemia myelofibrosis Post-essential thrombocythemia myelofibrosis Management recommendations Targeted therapies Janus kinase inhibitors Ruxolitinib 



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.

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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Copyright information

© Springer-Verlag Wien 2016

Authors and Affiliations

  • Thamer Sliwa
    • 1
    Email author
  • Christine Beham-Schmid
    • 2
  • Sonja Burgstaller
    • 3
  • Veronika Buxhofer-Ausch
    • 4
  • Günther Gastl
    • 5
  • Klaus Geissler
    • 6
  • Maria Krauth
    • 7
  • Peter Krippl
    • 8
  • Alois Lang
    • 9
  • Andreas Petzer
    • 10
  • Stefan Wöhrer
    • 11
  • Albert Wölfler
    • 12
  • Heinz Gisslinger
    • 7
  1. 1.3rd Medical DepartmentHanusch HospitalViennaAustria
  2. 2.Institute of PathologyMedical University of GrazGrazAustria
  3. 3.Department of Internal Medicine IVKlinikum Wels-GrieskirchenWelsAustria
  4. 4.Department of Internal Medicine IElisabethinen Hospital LinzLinzAustria
  5. 5.Division of Hematology and OncologyInnsbruck Medical UniversityInnsbruckAustria
  6. 6.5th Medical Department with Hematology, Oncology and Palliative MedicineHospital HietzingViennaAustria
  7. 7.Department of Internal Medicine I, Division of Hematology and Blood CoagulationMedical University of ViennaViennaAustria
  8. 8.Department of Internal Medicine with Hematology and OncologySteiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-FürstenfeldFürstenfeldAustria
  9. 9.Department of Internal MedicineLKH FeldkirchFeldkirchAustria
  10. 10.Department of Medical Oncology, Hematology and GastroenterologyBarmherzige Schwestern Hospital LinzLinzAustria
  11. 11.Department of OncologyLandesklinikum Wiener NeustadtWiener NeustadtAustria
  12. 12.Division of HematologyMedical University of GrazGrazAustria

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