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Erschienen in: Wiener klinische Wochenschrift 9-10/2017

01.05.2017 | consensus report

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

verfasst von: Thamer Sliwa, 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

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 9-10/2017

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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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Metadaten
Titel
Austrian recommendations for the management of primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis: an expert statement
verfasst von
Thamer Sliwa
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
Publikationsdatum
01.05.2017
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 9-10/2017
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-016-1120-8