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- W2029944177 abstract "SUMMARY Myeloproliferative neoplasms (MPNs) that do not harbor the BCR–ABL rearrangement include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis. All of these diseases are characterized by an increased risk of vascular complications and by the propensity to evolve into acute leukemia. The JAK2V617F mutation determines a gain of function in the gene encoding JAK2 and is the most frequent molecular abnormality in MPNs, with an estimated prevalence of more than 95% in PV and 50% in ET and primary myelofibrosis. Molecular markers, together with marrow histology and cytogenetic data, are increasingly relevant for MPN diagnosis, and their prognostic value is under evaluation. In PV and ET, the use of aspirin, hydroxyurea and phlebotomy remain the mainstay of treatment. In myelofibrosis, conventional therapy (androgens, steroids, chemotherapy and splenectomy) has still only palliative effects. The only potentially curative approach is allogeneic stem cell transplantation, but treatment-related mortality remains high. In the last 2 years, the JAK–STAT pathway has become the target of selective tyrosine kinase inhibitors, which might represent a promising therapeutic option. Their role in future therapy, as single agents and/or in combinatorial approaches, is yet to be determined." @default.
- W2029944177 created "2016-06-24" @default.
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- W2029944177 date "2013-06-01" @default.
- W2029944177 modified "2023-09-27" @default.
- W2029944177 title "Update on the treatment of Ph-negative myeloproliferative neoplasms" @default.
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- W2029944177 doi "https://doi.org/10.2217/ijh.13.24" @default.
- W2029944177 hasPublicationYear "2013" @default.
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