Myeloproliferative neoplasms: from origins to outcomes.
Hematology Am Soc Hematol Educ Program
American Society of Hematology
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Nangalia, J., & Green, T. (2017). Myeloproliferative neoplasms: from origins to outcomes.. Hematology Am Soc Hematol Educ Program, 2017 (1), 470-479. https://doi.org/10.1182/asheducation-2017.1.470
Substantial progress has been made in our understanding of the pathogenetic basis of myeloproliferative neoplasms. The discovery of mutations in JAK2 over a decade ago heralded a new age for patient care as a consequence of improved diagnosis and the development of therapeutic JAK inhibitors. The more recent identification of mutations in calreticulin brought with it a sense of completeness, with most patients with myeloproliferative neoplasm now having a biological basis for their excessive myeloproliferation. We are also beginning to understand the processes that lead to acquisition of somatic mutations and the factors that influence subsequent clonal expansion and emergence of disease. Extended genomic profiling has established a multitude of additional acquired mutations, particularly prevalent in myelofibrosis, where their presence carries prognostic implications. A major goal is to integrate genetic, clinical, and laboratory features to identify patients who share disease biology and clinical outcome, such that therapies, both existing and novel, can be better targeted.
Animals, Humans, Hematologic Neoplasms, Myeloproliferative Disorders, Protein Kinase Inhibitors, DNA Mutational Analysis, Myelopoiesis, Mutation, Janus Kinase 2
External DOI: https://doi.org/10.1182/asheducation-2017.1.470
This record's URL: https://www.repository.cam.ac.uk/handle/1810/280478