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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
- 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.
- Subjects :
- Oncology
medicine.medical_specialty
Ruxolitinib
Disease
03 medical and health sciences
0302 clinical medicine
Polycythemia vera
hemic and lymphatic diseases
Internal medicine
Myeloproliferation
medicine
Molecular Targeted Therapy
Leukocytosis
Myelofibrosis
Expert Testimony
Thrombocytosis
business.industry
Antibodies, Monoclonal
Hematology
General Medicine
medicine.disease
Transplantation
Primary Myelofibrosis
Austria
030220 oncology & carcinogenesis
Practice Guidelines as Topic
Immunology
medicine.symptom
business
030215 immunology
medicine.drug
Subjects
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