title: Austrian recommendations for the management of primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis: an expert statement.
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.
authors.affiliation: 3rd Medical Department, Hanusch Hospital, Vienna, Austria. thamer.sliwa@wgkk.at.
authors.forename: Thamer
authors.initials: T
authors.lastname: Sliwa
authors.affiliation: Institute of Pathology, Medical University of Graz, Graz, Austria.
authors.forename: Christine
authors.initials: C
authors.lastname: Beham-Schmid
authors.affiliation: Department of Internal Medicine IV, Klinikum Wels-Grieskirchen, Wels, Austria.
authors.forename: Sonja
authors.initials: S
authors.lastname: Burgstaller
authors.affiliation: Department of Internal Medicine I, Elisabethinen Hospital Linz, Linz, Austria.
authors.forename: Veronika
authors.initials: V
authors.lastname: Buxhofer-Ausch
authors.affiliation: Division of Hematology and Oncology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Günther
authors.initials: G
authors.lastname: Gastl
authors.affiliation: 5th Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Vienna, Austria.
authors.forename: Klaus
authors.initials: K
authors.lastname: Geissler
authors.affiliation: Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria.
authors.forename: Maria
authors.initials: M
authors.lastname: Krauth
authors.affiliation: Department of Internal Medicine with Hematology and Oncology, Steiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-Fürstenfeld, Fürstenfeld, Austria.
authors.forename: Peter
authors.initials: P
authors.lastname: Krippl
authors.affiliation: Department of Internal Medicine, LKH Feldkirch, Feldkirch, Austria.
authors.forename: Alois
authors.initials: A
authors.lastname: Lang
authors.affiliation: Department of Medical Oncology, Hematology and Gastroenterology, Barmherzige Schwestern Hospital Linz, Linz, Austria.
authors.forename: Andreas
authors.initials: A
authors.lastname: Petzer
authors.affiliation: Department of Oncology, Landesklinikum Wiener Neustadt, Wiener Neustadt, Austria.
authors.forename: Stefan
authors.initials: S
authors.lastname: Wöhrer
authors.affiliation: Division of Hematology, Medical University of Graz, Graz, Austria.
authors.forename: Albert
authors.initials: A
authors.lastname: Wölfler
authors.affiliation: Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria.
authors.forename: Heinz
authors.initials: H
authors.lastname: Gisslinger
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D001317:Austria; D005104:Expert Testimony; D006405:Hematology / Q000592:standards*; D058990:Molecular Targeted Therapy / Q000379:methods*; D017410:Practice Guidelines as Topic; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*
chemical_list: D000911:Antibodies, Monoclonal
keywords: Janus kinase inhibitors; Management recommendations; Post-essential thrombocythemia myelofibrosis; Post-polycythemia myelofibrosis; Primary myelofibrosis; Ruxolitinib; Targeted therapies