Mutated JAK2 signal transduction in human induced pluripotent stem cell (iPSC)-derived megakaryocytes

Pawinwongchai, Jaturawat; Jangprasert, Panchalee; Nilsri, Nungruthai; Israsena, Nipan; Rojnuckarin, Ponlapat

doi:10.1080/09537104.2021.1981850

Cited by 1 publication

(1 citation statement)

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“… 65 The study of JAK2 V617F pathophysiology has yielded many new candidates to target clonal expansion in PV. Therapies that upregulate hepcidin, 90 suppress key inflammatory signaling pathways, 26 , 91 , 92 activate p53, 93 , 94 inhibit HIF, 95 target DNA repair pathways, 30 , 96 or facilitate JAK2 V617F stem cell extinction 97 , 98 are exciting developments for PV and will complement JAK2 inhibitor and interferon alfa–based therapies. Our clinical and research goals should be to incorporate these new targets in the treatment of PV, to use the knowledge of JAK2 V617F VAF to better prognosticate and monitor clonal dynamics, and to aspire to provide reliable, safe, and timely clonal suppression ( Table 5 ).…”

Section: Discussionmentioning

confidence: 99%

JAK2 V617F allele burden in polycythemia vera: burden of proof

Moliterno

Kaizer

Reeves

2023

Blood

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Polycythemia vera (PV) is a hematopoietic stem cell (HSC) neoplasm defined by activating somatic mutations in the JAK2 gene and characterized clinically by overproduction of red blood cells, platelets, and neutrophils; significant burden of disease-specific symptoms; high rates of vascular events; and evolution to a myelofibrosis phase or acute leukemia. The JAK2V617F variant allele frequency (VAF) is a key determinant of outcomes in PV, including thrombosis and myelofibrotic progression. Herein we critically review the dynamic role of JAK2V617F mutation burden in the pathogenesis and natural history of PV, suitability of JAK2V617F VAF as a diagnostic and prognostic biomarker, and utility of JAK2V617F VAF reduction in PV treatment.

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