MPN: The Molecular Drivers of Disease Initiation, Progression and Transformation and their Effect on Treatment

Julian Grabek, Jasmin Straube, Megan Bywater, Steven W. Lane
2020 Cells  
Myeloproliferative neoplasms (MPNs) constitute a group of disorders identified by an overproduction of cells derived from myeloid lineage. The majority of MPNs have an identifiable driver mutation responsible for cytokine-independent proliferative signalling. The acquisition of coexisting mutations in chromatin modifiers, spliceosome complex components, DNA methylation modifiers, tumour suppressors and transcriptional regulators have been identified as major pathways for disease progression and
more » ... leukemic transformation. They also confer different sensitivities to therapeutic options. This review will explore the molecular basis of MPN pathogenesis and specifically examine the impact of coexisting mutations on disease biology and therapeutic options.
doi:10.3390/cells9081901 pmid:32823933 fatcat:k5govrwwevbpngsstnzr5iq4f4