title: Blast-phase myeloproliferative neoplasms: risk factors and treatment approaches.
abstract: Introduction:The past 10 years have seen dramatic advances in the understanding of the molecular pathogenesis ofBCR-ABLnegative myeloproliferative neoplasms (MPNs). With this knowledge has come novel, molecularly targeted therapies such as JAK inhibitors that may decrease symptoms and improve quality of life for patients with MPNs. Despite these advances, progression of the disease to an acute leukemic (blast) phase remains difficult to predict and even more difficult to treat, with high rates of disease relapse and mortality.Areas covered:We performed a literature review of known risk factors for progression of MPNs towards blast phase and treatment options for transformed disease, including approved and investigational agents. Herein, we review the current literature and suggest strategies for improving outcomes in the future.Expert commentary:Further understanding of the biologic basis for transformation of MPNs from the chronic to blast phase is needed in order to predict, prevent, and treat these cases. Patients with MPNs in blast phase should be encouraged to participate in clinical trials whenever possible.
authors.affiliation: Department of Medicine, Section of Hematology/Oncology, University of Chicago, Chicago, IL, USA.|Committee on Clinical Pharmacology and Pharmacogenomics, University of Chicago, Chicago, IL, USA.
authors.forename: Kristen
authors.initials: K
authors.lastname: Pettit
authors.affiliation: Department of Medicine, Section of Hematology/Oncology, University of Chicago, Chicago, IL, USA.|University of Chicago Comprehensive Cancer Center, Chicago, IL, USA.
authors.forename: Olatoyosi
authors.initials: O
authors.lastname: Odenike
keywords: Myeloproliferative neoplasms; blast-phase; hypomethylating agents; novel therapies; ruxolitinib; stem cell transplantation