SOHO state-of-the-art update and next questions: MPN

Publication date: Available online 2 December 2017 Source:Clinical Lymphoma Myeloma and Leukemia Author(s): Prithviraj Bose, Jason Gotlib, Claire N. Harrison, Srdan Verstovsek The discovery of the activating JAK2 V617F mutation in 2005 in the majority of patients with the classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) spurred intense interest in research into these disorders, culminating in the identification of activating mutations in MPL in 2006 and indels in CALR in 2013, thus providing additional mechanistic explanations for the universal activation of Janus kinase-signal transducer and activator of transcription (JAK-STAT) observed in these conditions, and the success of the JAK1/2 inhibitor ruxolitinib, which first received regulatory approval in 2011. The field has continued to advance rapidly since then, and the last two years have witnessed important changes to the classification of MPN and diagnostic criteria for polycythemia vera (PV), novel insights into the mechanisms of bone marrow fibrosis in primary myelofibrosis (PMF), increasing appreciation of the biologic differences between essential thrombocythemia (ET), prefibrotic and overt PMF and between primary and post-PV/ET myelofibrosis (MF). Additionally, the mechanisms through which mutant calreticulin drives JAK-STAT pathway activation and oncogenic transformation are now better understood. Although mastocytosis is no longer included under the broad heading of MPN in the 2016...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research