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- Title
Selective JAK/STAT Inhibition: Signaling a Way Forward in the Management of Myelofibrosis.
- Authors
Danaee, Anicee; Harrison, Claire
- Abstract
Primary Myelofibrosis (PMF) is the rarest of all myeloproliferative neoplasms (MPN) and remains a devastating disease. Bone marrow transplant offers the only possibility of cure, however, it is not a realistic therapeutic option for the majority of patients with PMF who tend to present later in life and with comorbidities. In the last few years, the discovery of JAK2V617F and the subsequent development of JAK2 inhibitors mainly ruxolitinib, with many other agents at different stages of development, has been an important step forward in the management of constitutional symptoms and splenomegaly in these patients, and more importantly, there seems to be increasing evidence of its long-term benefits for survival. Concerning pathogenesis of MPN involvement of the JAK-STAT pathway, however, is only a part of the puzzle; increasingly the role of epigenetics in MPN is being recognized, and current trials are looking at the combination therapies with the aim to either minimize side effects of anemia and thrombocytopenia or deepen disease benefit, for example, the concomitant use of histone deacetylase inhibitors (HDACi). In spite of the progress that has been made in prognostication and management of MPNs, there remain several challenges including overcoming resistance to JAK2 inhibitors and impacting upon underlying disease biology as exemplified by many clinical features including bone marrow fibrosis.
- Publication
Journal of OncoPathology, 2014, Vol 2, Issue 3, p45
- ISSN
2052-5931
- Publication type
Academic Journal
- DOI
10.13032/tjop.2052-5931.100092