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Annual Review of Pathology: Mechanisms of Disease

Volume 11, 2016

Pathogenesis of Myeloproliferative Disorders

Abstract

Myeloproliferative neoplasms (MPNs) are a set of chronic hematopoietic neoplasms with overlapping clinical and molecular features. Recent years have witnessed considerable advances in our understanding of their pathogenetic basis. Due to their protracted clinical course, the evolution to advanced hematological malignancies, and the accessibility of neoplastic tissue, the study of MPNs has provided a window into the earliest stages of tumorigenesis. With the discovery of mutations in , the majority of MPN patients now bear an identifiable marker of clonal disease; however, the mechanism by which mutated CALR perturbs megakaryopoiesis is currently unresolved. We are beginning to understand better the role of homozygosity, the function of comutations in epigenetic regulators and spliceosome components, and how these mutations cooperate with to modulate MPN phenotype.

Keyword(s): CALR, epigenetic, JAK2, MPL, myeloproliferative neoplasms
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2016-05-23
2024-04-19
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Pathogenesis of Myeloproliferative Disorders
Annual Review of Pathology: Mechanisms of Disease 11, 101 (2016); https://doi.org/10.1146/annurev-pathol-012615-044454
/content/journals/10.1146/annurev-pathol-012615-044454
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