Myeloproliferative neoplasms in 2022
A concise review
DOI:
https://doi.org/10.58931/cht.2022.117Abstract
The Philadelphia chromosome(Ph)-negative myeloproliferative neoplasms (MPN) are comprised of a heterogenous group of disorders of myeloid hematopoietic stem cells that include polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (MF). MPN are characterized by constitutional and other disease-related symptoms, an increased risk for thrombotic and hemorrhagic events, and a propensity to transform to acute myeloid leukemia (AML). Progress in our understanding of the molecular pathophysiology of MPN has led to improved prognostic tools, and increasingly personal risk-stratification. In PV, there has been renewed interest in interferon (IFN) for its potential to directly target the malignant clone and exert a disease-modifying effect. In MF, the introduction of Janus Kinase (JAK) inhibitors has significantly altered the therapeutic landscape over the past decade. Ongoing development in the area of JAK inhibitor therapy, as well as several novel pathways, holds promise for improved hematologic responses, lessening of overall burden of illness, increased quality of life, and application to a broader cohort of patients.
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