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

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

Authors :
Maria Theresa Krauth
Andreas L. Petzer
Sonja Burgstaller
Veronika Buxhofer-Ausch
Stefan Wöhrer
Christine Beham-Schmid
Günther Gastl
Alois Lang
Heinz Gisslinger
Albert Wölfler
Thamer Sliwa
Klaus Geissler
Peter Krippl
Source :
Wiener klinische Wochenschrift. 129:293-302
Publication Year :
2016
Publisher :
Springer Science and Business Media LLC, 2016.

Abstract

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.

Details

ISSN :
16137671 and 00435325
Volume :
129
Database :
OpenAIRE
Journal :
Wiener klinische Wochenschrift
Accession number :
edsair.doi.dedup.....768b2dcccdccc2f2c472044657896471
Full Text :
https://doi.org/10.1007/s00508-016-1120-8