PV begins in the bone marrow, with a mutation in hematopoietic stem cells (HSCs)1
In 97% of cases, the JAK2 mutation drives pathogenesis2
Mutated HSCs acquire a selective advantage that favors their proliferation over normal HSCs1Mutated stem cell proliferation leaves your patients at risk
PV begins in the bone marrow, with a mutation in hematopoietic stem cells (HSCs)1 In 97% of cases, the JAK2 mutation drives pathogenesis2Mutated HSCs acquire a selective advantage that favors their proliferation over normal HSCs1In PV, the mutated HSCs drive overproduction of red blood cells (RBCs), white blood cells (WBCs), and platelets (PLTs). Elevated RBCs, WBCs, and PLT counts are each associated with increased thrombotic risk.3,4
Indicators of disease progression include:
Over time the root cause of PV, the mutated stem cells, continues to proliferate putting patients at risk.1,4,7
Slow/delay progression of conditionWas patients’ top treatment goal, according to the MPN Landmark survey8
When evaluating treatment strategies, HCPs should consider10:
There is now a growing interest in JAK2 (V617F) allele burden and its potential influence on disease phenotype, disease complications, and evolution —Passamonti F, et al11
The JAK2 V617F allele burden—or the proportion of mutant cells to healthy cells—is an increasingly studied concept that may affect how we think about PV.9,11
Quantifying the mutation may be important because allele burden can be used to measure clonal expansion, and can also provide insight into disease progression.9,11
Higher allele burden may be associated with:
The clinical benefit of reducing JAK2 allelic burden is still an ongoing area of scientific interest and prospective validation is needed.
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References: 1. Mead AJ, Mullally A. Myeloproliferative neoplasm stem cells. Blood. 2017;129(12); 1607-1616. 2. Tefferi A, Vannucchi AM, Barbui T. Polycythemia vera treatment algorithm 2018. Blood Cancer J. 2018;8(1):3. 3. Stein BL, Oh ST, Berenzon D, et al. Polycythemia vera: an appraisal of the biology and management 10 years after the discovery of JAK2 V617F. J Clin Oncol. 2015;33(33):3953-3960. 4. Passamonti F, Rumi E, Pietra D, et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia. 2010;24(9):1574-1579. 5. Waggoner M. Polycythemia vera: thinking beyond the hematocrit. J Adv Pract Oncol. 2023;14(5):405-413. 6. Fox S, Griffin L, Harris DR. Polycythemia vera: rapid evidence review. Am Fam Physician. 2021;103(11):680-687. 7. Libby P, Sidlow R, Lin AE, et al. Clonal hematopoiesis: crossroads of aging, cardiovascular disease, and cancer: JACC review topics of the week. J Am Coll Cardiol. 2019;74(4):567-577. 8. Mesa R, Miller CB, Thyne M, et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016;16:167. 9. Moliterno AR, Kaizer H, Reeves BN. JAK2 V617F allele burden in polycythemia vera: burden of proof. Blood. 2023;141(16):1934-1942. 10. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Myeloproliferative Neoplasms V.1.2024. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed December 21, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 11. Passamonti F, Rumi E. Clinical relevance of JAK2 (V617F) mutant allele burden. Haematologica. 2009;94(1):7-10. 12. Vannucchi AM, Antonioli E, Guglielmelli P, et al. Prospective identification of high-risk polycythemia vera patients based on JAK2 (V617F) allele burden. Leukemia. 2007;21(9):1952-1959.