Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Sliwa, T; Beham-Schmid, C; Burgstaller, S; Buxhofer-Ausch, V; Gastl, G; Geissler, K; Krauth, M; Krippl, P; Lang, A; Petzer, A; Wöhrer, S; Wölfler, A; Gisslinger, H.
Austrian recommendations for the management of primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis: an expert statement.
Wien Klin Wochenschr. 2017; 129(9-10):293-302
Doi: 10.1007/s00508-016-1120-8
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- Co-Autor*innen der Med Uni Graz
- Beham-Schmid Christine
- Krippl Peter
- Wölfler Albert
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Antibodies, Monoclonal - therapeutic use
- Austria -
- Expert Testimony -
- Hematology - standards
- Molecular Targeted Therapy - methods
- Practice Guidelines as Topic -
- Primary Myelofibrosis - diagnosis
- Primary Myelofibrosis - pathology
- Primary Myelofibrosis - therapy
- Find related publications in this database (Keywords)
- Primary myelofibrosis
- Post-polycythemia myelofibrosis
- Post-essential thrombocythemia myelofibrosis
- Management recommendations
- Targeted therapies
- Janus kinase inhibitors
- Ruxolitinib